LEPRA Y FENOMENO DE LUCIO. !
LEPROSY AND THE LUCIO'S PHENOMENON. !
ULCERAS EN MIEMBROS INFERIORES PACIENTE CON LEPRA
EDITORIAL ESPAÑOL
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Hola amigos de la red. DERMAGIC de nuevo con ustedes, el tema de hoy: EL FENÓMENO DE LUCIO llamado también ERITEMA NECROTIZANTE.
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Hola amigos de la red. DERMAGIC de nuevo con ustedes, el tema de hoy: EL FENÓMENO DE LUCIO llamado también ERITEMA NECROTIZANTE.
HISTORIA:
Es uno de los tipos de reacción leprosa (Reacción tipo II) , poco
común y descrito por primera vez por
RAFAEL LUCIO NÁJERA, médico mejicano e IGNACIO ALVARADO, también Mejicano, en 1.852
como una reacción cutánea necrosante asociada con lepra difusa no
nodular anergica, o en pacientes con tratamientos incompletos.
En el año 1936 JOSE FERNANDO LATAPI, también Dermatólogo mejicano describió esta manifestación de la
Lepra difusa, y años después el mismo
FERNANDO LATAPI y AGUSTIN ZAMORA
en 1.948 lo documentaron microscópicamente, y lo denominaron
definitivamente FENÓMENO DE LUCIO, o
LEPRA DE LUCIO-LATAPI.
CARACTERÍSTICAS CLÍNICAS:
Se presenta principalmente en la lepra lepromatosa (LL) difusa
llamada también lepra de LUCIO y LATAPI, caracterizada
principalmente por la
ausencia de NÓDULOS en la piel. Se trata de una reacción leprosa grave y poco frecuente en estos
pacientes de LEPRA LEPROMATOSA (LL) DIFUSA, no tratada o con
tratamiento incompleto.
Este tipo de lepra es bastante común en
Centro América y México.
Las LESIONES CARACTERÍSTICAS
SON ÚLCERAS las cuales
inicialmente se presentan como
máculas purpúricas, que evolucionan posteriormente a lesiones ulcerosas
superficiales, de formas poligonales o anguladas, posteriormente
quedan lesiones cicatriciales atroficas, en forma de estrella.
Las lesiones se presentan principalmente miembros inferiores, pero otras partes del cuerpo pueden estar involucradas, como cuero cabelludo, cara y miembros superiores, pero no tan frecuentemente.
Hay sensación de quemazón, pero NO HAY, fiebre, tampoco síntomas
sistémicos marcados.
Como dijimos, se presenta en pacientes con
ALTA CARGA BACILAR, (lepra lepromatosa difusa), y puede ser potencialmente
mortal, si no se instaura un tratamiento oportuno.
BIOPSIA:
Histopatológicamente hoy día es considerado como una
PANVASCULITIS NECROTIZANTE, caracterizada por necrosis de la epidermis y vasos
sanguíneos en la dermis, ocasionada por una oclusión de los mismos,
la cual provoca inflamación vascular mediada por
inmunocomplejos.
INMUNOLOGÍA:
EL FENÓMENO DE LUCIO esta clasificado dentro de las reacciones
leprosas como
UNA REACCIÓN TIPO 2: la cual es una reacción inmunológica la cual esta
mediada por complejos inmunes, los cuales provocan inflamación severa de los vasos
sanguíneos, desencadenando trombosis y necrosis, que se manifiesta
con las lesiones antes mencionadas.
Esta REACCIÓN TIPO 2, también se presenta en el ERITEMA NODOSO
LEPROSO, pero con variante clínica e histopatológica diferente.
El FENÓMENO DE LUCIO ES muy común en los pacientes con lepra difusa
en México, recientemente se le ha descrito asociado al nuevo agente
causal de la LEPRA descrito en 2.008 el
MYCOBATERIUM LEPROMATOSUS, en algunos casos se ha encontrado asociación con el Síndrome de
anticuerpos ANTIFOSFOLIPIDOS (anticardiolipinas), y es considerado
la reacción mas anérgica del espectro de la lepra lepromatosa (LL)
difusa.
TRATAMIENTO:
1.- Terapia Multi-droga (MTD):
Tratamienco clasico de la LEPRA: Diaminodifenil sulfona (DDS),
RIFAMPICINA y CLOFAZIMINE (Lampren)
2.- Talidomida: Es un agente inmunomodulador el cual se usa en combinación
con corticosteroides orales, para controlar la
VASCULITIS NECROTIZANTE.
3.- Esteroides:
Prednisolona o prednisona a altas dosis, para combatir la
inflamación.
4.- Antibióticoterapia: Con buenos resultados, para tratar infección secundaria o
prevenirla, con buenos resultados.
5.- Anticoagulantes y vasodilatadores:
para evitar la formación de coágulos y mejorar la
circulación.
6.- Hospitalización: Para monitoreo constante del paciente, ya que en algunos
casos el desenlace puede ser fatal
7.- Medidas de soporte:
tratamiento local de las ulceras y el dolor.
CONCLUSIONES:
El FENÓMENO DE LUCIO, es una manifestación cutánea, que se presenta
por lo general en pacientes con LEPRA LEPROMATOSA DIFUSA a nivel de
miembros inferiores, caracterizada por ULCERAS.
Inmunógicamente es una REACCIÓN LEPROSA TIPO 2, provocada por
complejos inmunes, muy común en MEJICO, CENTRO (Costa Rica) y
SUR-AMERICA, también se ha descrito en países no endémicos para esta
patología como CANADA; puede haber desenlace fatal si no es tratada
a tiempo.
El FENÓMENO DE LUCIO es considerado la reacción más anérgica de todo el espectro inmunológico de la
lepra.
Como dato HISTÓRICO el mismo RAFAEL LUCIO NÁJERA, se inoculo a si mismo con este tipo de lepra de la que murió en
1886, a los 66 años.
Hoy, en nuestros días, EL FENÓMENO DE LUCIO es una realidad
en el mundo de la LEPRA.
En las 65 referencias los hechos, en la foto: CLÁSICO FENÓMENO DE
LUCIO, y biopsia de lesiones.
Saludo a todos.
Dr. José Lapenta.
Dr. José M. Lapenta.
EDITORIAL ENGLISH
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Hello friends of the network. DERMAGIC is back with you again, today's
topic: LUCIO'S PHENOMENON, also known as NECROTIZING ERYTHEMA.
HISTORY:
It is one of the types of leprous reactions (Type II reaction), rare and first described by RAFAEL LUCIO NÁJERA, a Mexican doctor, and IGNACIO ALVARADO, also Mexican, in 1852 as a necrotizing cutaneous reaction associated with diffuse non-nodular anergic leprosy, or in patients with incomplete treatment.
In 1936, JOSE FERNANDO LATAPI ZAMORA, also a Mexican dermatologist, described this manifestation of diffuse leprosy. Years later, FERNANDO LATAPI and AGUSTIN ZAMORA documented it microscopically in 1948, definitively naming it LUCIO'S PHENOMENON, or LEPROSY OF LUCIO-LATAPI.
CLINICAL CHARACTERISTICS:
It occurs primarily in diffuse Lepromatous Leprosy (LL), also known as LUCIO and LATAPI leprosy, characterized primarily by the absence of skin nodules. It is a severe and rare leprous reaction in patients with untreated or incompletely treated DIFFUSE LEPROMATOUS LEPROSY (LL).
This type of leprosy is quite common in CENTRAL AMERICA and MEXICO. The CHARACTERISTIC LESIONS ARE ULCERS, which initially present as purpuric macules, which later evolve into superficial ulcerative lesions with polygonal or angular shapes, and subsequently become atrophic, star-shaped scarring lesions.
The lesions primarily occur on the LOWER LIMBS, but other parts of the body may be involved, such as the scalp, face, and upper limbs, but not as frequently.
There is a burning sensation, but NO fever, nor any marked systemic symptoms.
As mentioned, it occurs in patients with a HIGH bacillary load (diffuse lepromatous leprosy) and can be life-threatening if not treated promptly.
BIOPSY:
Histopathologically, it is currently considered NECROTIZING PANVASCULITIS, characterized by necrosis of the epidermis and blood vessels in the dermis, caused by their occlusion, which causes immune-complex-mediated vascular inflammation.
IMMUNOLOGY:
LUCIO'S PHENOMENON is classified within leprosy reactions as a TYPE 2 REACTION: an immunological reaction mediated by immune complexes, which causes severe inflammation of the blood vessels, triggering thrombosis and necrosis, manifesting with the aforementioned lesions.
This TYPE 2 REACTION also occurs in ERYTHEMA NODOSUM LEPROSUM, but with a different clinical and histopathological variant.
LUCIO'S PHENOMENON IS very common in patients with diffuse leprosy in Mexico. It has recently been described in association with the new causative agent of leprosy, MYCOBATERIUM LEPROMATOSUS, described in 2008. In some cases, an association with ANTIPHOSPHOLIPID ANTIBODY SYNDROME (anticardiolipins) has been found. It is considered the most anergic reaction in the spectrum of diffuse lepromatous leprosy (LL).
HISTORY:
It is one of the types of leprous reactions (Type II reaction), rare and first described by RAFAEL LUCIO NÁJERA, a Mexican doctor, and IGNACIO ALVARADO, also Mexican, in 1852 as a necrotizing cutaneous reaction associated with diffuse non-nodular anergic leprosy, or in patients with incomplete treatment.
In 1936, JOSE FERNANDO LATAPI ZAMORA, also a Mexican dermatologist, described this manifestation of diffuse leprosy. Years later, FERNANDO LATAPI and AGUSTIN ZAMORA documented it microscopically in 1948, definitively naming it LUCIO'S PHENOMENON, or LEPROSY OF LUCIO-LATAPI.
CLINICAL CHARACTERISTICS:
It occurs primarily in diffuse Lepromatous Leprosy (LL), also known as LUCIO and LATAPI leprosy, characterized primarily by the absence of skin nodules. It is a severe and rare leprous reaction in patients with untreated or incompletely treated DIFFUSE LEPROMATOUS LEPROSY (LL).
This type of leprosy is quite common in CENTRAL AMERICA and MEXICO. The CHARACTERISTIC LESIONS ARE ULCERS, which initially present as purpuric macules, which later evolve into superficial ulcerative lesions with polygonal or angular shapes, and subsequently become atrophic, star-shaped scarring lesions.
The lesions primarily occur on the LOWER LIMBS, but other parts of the body may be involved, such as the scalp, face, and upper limbs, but not as frequently.
There is a burning sensation, but NO fever, nor any marked systemic symptoms.
As mentioned, it occurs in patients with a HIGH bacillary load (diffuse lepromatous leprosy) and can be life-threatening if not treated promptly.
BIOPSY:
Histopathologically, it is currently considered NECROTIZING PANVASCULITIS, characterized by necrosis of the epidermis and blood vessels in the dermis, caused by their occlusion, which causes immune-complex-mediated vascular inflammation.
IMMUNOLOGY:
LUCIO'S PHENOMENON is classified within leprosy reactions as a TYPE 2 REACTION: an immunological reaction mediated by immune complexes, which causes severe inflammation of the blood vessels, triggering thrombosis and necrosis, manifesting with the aforementioned lesions.
This TYPE 2 REACTION also occurs in ERYTHEMA NODOSUM LEPROSUM, but with a different clinical and histopathological variant.
LUCIO'S PHENOMENON IS very common in patients with diffuse leprosy in Mexico. It has recently been described in association with the new causative agent of leprosy, MYCOBATERIUM LEPROMATOSUS, described in 2008. In some cases, an association with ANTIPHOSPHOLIPID ANTIBODY SYNDROME (anticardiolipins) has been found. It is considered the most anergic reaction in the spectrum of diffuse lepromatous leprosy (LL).
TREATMENT:
1.- Multi-drug therapy (MTD): Classic treatment for leprosy: Diaminodiphenyl sulfone (DDS), rifampicin, and clofazimine (Lampren).
2.- Thalidomide: This is an immunomodulatory agent used in combination with oral corticosteroids to control necrotizing vasculitis.
3.- Steroids: Prednisolone or prednisone at high doses to combat inflammation.
4.- Antibiotic therapy: This therapy has been successful in treating or preventing secondary infection.
5.- Anticoagulants and vasodilators: To prevent clot formation and improve circulation.
6.- Hospitalization: For constant patient monitoring, as in some cases the outcome can be fatal.
7.- Supportive measures: Local treatment of ulcers and pain.
CONCLUSIONS:
Lucio's phenomenon is a cutaneous manifestation that usually occurs in patients with diffuse lepromatous leprosy of the lower limbs, characterized by ulcers.
Immunologically, it is a type 2 leprous reaction, caused by immune complexes, very common in Mexico, Central America (Costa Rica), and South America. It has also been described in countries not endemic for this disease, such as Canada. It can be fatal if not treated promptly.
Lucio's phenomenon is considered the most anergic reaction in the entire immunologic spectrum of leprosy.
As a HISTORICAL fact, RAFAEL LUCIO NÁJERA inoculated himself with this type of leprosy, from which he died in 1886, at the age of 66.
Today, the LUCIO PHENOMENON is a reality in the world of leprosy.
The facts are described in the 65 references, in the photo: CLASSIC LUCIO PHENOMENON, and biopsy of lesions.
1.- Multi-drug therapy (MTD): Classic treatment for leprosy: Diaminodiphenyl sulfone (DDS), rifampicin, and clofazimine (Lampren).
2.- Thalidomide: This is an immunomodulatory agent used in combination with oral corticosteroids to control necrotizing vasculitis.
3.- Steroids: Prednisolone or prednisone at high doses to combat inflammation.
4.- Antibiotic therapy: This therapy has been successful in treating or preventing secondary infection.
5.- Anticoagulants and vasodilators: To prevent clot formation and improve circulation.
6.- Hospitalization: For constant patient monitoring, as in some cases the outcome can be fatal.
7.- Supportive measures: Local treatment of ulcers and pain.
CONCLUSIONS:
Lucio's phenomenon is a cutaneous manifestation that usually occurs in patients with diffuse lepromatous leprosy of the lower limbs, characterized by ulcers.
Immunologically, it is a type 2 leprous reaction, caused by immune complexes, very common in Mexico, Central America (Costa Rica), and South America. It has also been described in countries not endemic for this disease, such as Canada. It can be fatal if not treated promptly.
Lucio's phenomenon is considered the most anergic reaction in the entire immunologic spectrum of leprosy.
As a HISTORICAL fact, RAFAEL LUCIO NÁJERA inoculated himself with this type of leprosy, from which he died in 1886, at the age of 66.
Today, the LUCIO PHENOMENON is a reality in the world of leprosy.
The facts are described in the 65 references, in the photo: CLASSIC LUCIO PHENOMENON, and biopsy of lesions.
In these 65 references the facts...
ULCERS IN LOWER LIMBS IN PATIENT WITH LEPROSY
Greetings to all.
Dr. José Lapenta.
Dr. José M. Lapenta.
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REFERENCIAS BIBLIOGRÁFICAS / BIBLIOGRAPHICAL REFERENCES
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REFERENCIAS BIBLIOGRÁFICAS / BIBLIOGRAPHICAL REFERENCES
===================================================================
H.- Lucio's phenomenon in a non-endemic region: A case report
(2024).
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1.) Lepra type reactions
2.) Diffuse Painless Ulcerations
3.) [Lucio-Latapi leprosy and the Lucio phenomenon]
4.) [Lucio's leprosy].
5.) [22 years of leprosy: histopathology]
6.) Lucio's phenomenon.
7.) Lucio's phenomenon and diffuse nonnodular lepromatous leprosy.
8.) Lucio's phenomenon: a comparative histological study.
9.) [Diffuse lepromatous leprosy disclosed by cutaneous vasculitis. The Lucio phenomenon].
10.) Immunologic aspects of leprosy as related to leucocytic isoantibodies
and platelet aggregating factors.
11.) The role of protein malnutrition in the pathogenesis of ulcerative "Lazarine" leprosy.
12.) Dermal ultrastructure in leprosy.
13.) Lepromatous and tuberculoid leprosy: clinical presentation and cytokine responses.
14.) Leprosy (Hansen's disease) in South Dakota.
15.) [Virchowian Hansen's disease, Lucio's phenomenon, cryptococcosis].
16.) Erythema nodosum leprosum in Singapore.
17.) Epidermal keratinocyte Ia expression, Langerhans cell hyperplasia and lymphocytic infiltration in skin lesions of leprosy.
18.) Specific antigen and antibody to Mycobacterium leprae in the cryoprecipitate of a patient with Lucio phenomenon.
19.) In situ characterization of T lymphocyte subsets in the reactional states of leprosy.
20.) Ultrastructure of the dermal microvasculature in leprosy.
21.) Lucio's phenomenon: a comparative histological study.
22.) Serum macrophage migration inhibition activity in patients with leprosy.
23.) [Leprosy tests: diagnostic problems].
24.) Serum and tissue lysozyme in leprosy.
25.)[Reactional status of leprosy].
26.) Auricular chondritis as a rheumatologic manifestation of Lucio's phenomenon: clinical improvement after plasmapheresis.
27.) Contemplative immune mechanism of Lucio phenomenon and its global status.
28.) Plasma exchange therapy in Lucio's phenomenon.
29.) [Lepromatous leprosy with extensive ulcerations and cachexia. The Lucio phenomenon? Lazarine leprosy]?
30.) [2 cases of Lucio phenomenon in Paraguay].
31.) An unusual case of leprosy with pathological features common to Lucio's phenomenon.
32.) Primary diffuse lepromatous leprosy with erythema necrotisans (lucio phenomenon).
33.) The "Lucio phenomenon" in diffuse leprosy.
34.) [Macular leprosy of Lucio--antimalarials in leprotic reaction].
35.) [Dermatology in the Central American tropics. I. Lucio's spotted leprosy. Antimalarials in the leprous reaction].
36.) [Lucio's leprosy].
37.) [Lucio phenomenon in leprosy reactions].
38.) Lucio's phenomenon: an overview.
39.) Lucio's phenomenon: an immune complex deposition syndrome in lepromatous leprosy.
40.) [Lucio's leprosy].
41.) Antiphospholipid antibodies thrombotic syndrome misdiagnosed as Lucio's phenomenon.
================================
50.) Lucio's phenomenon is a necrotizing panvasculitis: mostly a medium-sized granulomatous arteritis.
================================
========================================================================
2.) Diffuse Painless Ulcerations
3.) [Lucio-Latapi leprosy and the Lucio phenomenon]
4.) [Lucio's leprosy].
5.) [22 years of leprosy: histopathology]
6.) Lucio's phenomenon.
7.) Lucio's phenomenon and diffuse nonnodular lepromatous leprosy.
8.) Lucio's phenomenon: a comparative histological study.
9.) [Diffuse lepromatous leprosy disclosed by cutaneous vasculitis. The Lucio phenomenon].
10.) Immunologic aspects of leprosy as related to leucocytic isoantibodies
and platelet aggregating factors.
11.) The role of protein malnutrition in the pathogenesis of ulcerative "Lazarine" leprosy.
12.) Dermal ultrastructure in leprosy.
13.) Lepromatous and tuberculoid leprosy: clinical presentation and cytokine responses.
14.) Leprosy (Hansen's disease) in South Dakota.
15.) [Virchowian Hansen's disease, Lucio's phenomenon, cryptococcosis].
16.) Erythema nodosum leprosum in Singapore.
17.) Epidermal keratinocyte Ia expression, Langerhans cell hyperplasia and lymphocytic infiltration in skin lesions of leprosy.
18.) Specific antigen and antibody to Mycobacterium leprae in the cryoprecipitate of a patient with Lucio phenomenon.
19.) In situ characterization of T lymphocyte subsets in the reactional states of leprosy.
20.) Ultrastructure of the dermal microvasculature in leprosy.
21.) Lucio's phenomenon: a comparative histological study.
22.) Serum macrophage migration inhibition activity in patients with leprosy.
23.) [Leprosy tests: diagnostic problems].
24.) Serum and tissue lysozyme in leprosy.
25.)[Reactional status of leprosy].
26.) Auricular chondritis as a rheumatologic manifestation of Lucio's phenomenon: clinical improvement after plasmapheresis.
27.) Contemplative immune mechanism of Lucio phenomenon and its global status.
28.) Plasma exchange therapy in Lucio's phenomenon.
29.) [Lepromatous leprosy with extensive ulcerations and cachexia. The Lucio phenomenon? Lazarine leprosy]?
30.) [2 cases of Lucio phenomenon in Paraguay].
31.) An unusual case of leprosy with pathological features common to Lucio's phenomenon.
32.) Primary diffuse lepromatous leprosy with erythema necrotisans (lucio phenomenon).
33.) The "Lucio phenomenon" in diffuse leprosy.
34.) [Macular leprosy of Lucio--antimalarials in leprotic reaction].
35.) [Dermatology in the Central American tropics. I. Lucio's spotted leprosy. Antimalarials in the leprous reaction].
36.) [Lucio's leprosy].
37.) [Lucio phenomenon in leprosy reactions].
38.) Lucio's phenomenon: an overview.
39.) Lucio's phenomenon: an immune complex deposition syndrome in lepromatous leprosy.
40.) [Lucio's leprosy].
41.) Antiphospholipid antibodies thrombotic syndrome misdiagnosed as Lucio's phenomenon.
42.) "Lucio's Phenomenon" Associated with Mycobacterium
lepromatosis.
43.) Lucio's phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka.
44.) Insight into the evolution and origin of leprosy bacilli from the genome sequence of Mycobacterium lepromatosis.
45.) Lucio Leprosy with Lucio's phenomenon, digital gangrene and anticardiolipin antibodies.
46.) Lucio's leprosy: a clinical and therapeutic challenge.
47.) Lucio's phenomenon: report of five cases.
48.) Severe leprosy reactions due to Mycobacterium lepromatosis.
49.) [Lucio's phenomenon in a patient with leprosy on Aruba].
50.) Lucio's phenomenon is a necrotizing panvasculitis: mostly a medium-sized granulomatous
arteritis.
51.) Livedo reticularis in type 2 lepra reaction: A rare presentation.
52.) Is it Lucio Phenomenon or Necrotic Erythema Nodosum Leprosum?
53.) Concomitant lucio phenomenon and erythema nodosum in a leprosy patient: clues for their distinct pathogeneses.
54.) Lucio's phenomenon. Case study of an exceptional response to treatment exclusively with
multibacillary multidrug therapy.
55.) [Lucio-Latapí leprosy and the Lucio phenomenon].
56.) Unusual Presentation of Necrotic Erythema Nodosum Leprosum on Scalp: A Case Report.
57.) Lucio's phenomenon: another case reported in Brazil.
43.) Lucio's phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka.
44.) Insight into the evolution and origin of leprosy bacilli from the genome sequence of Mycobacterium lepromatosis.
45.) Lucio Leprosy with Lucio's phenomenon, digital gangrene and anticardiolipin antibodies.
46.) Lucio's leprosy: a clinical and therapeutic challenge.
47.) Lucio's phenomenon: report of five cases.
48.) Severe leprosy reactions due to Mycobacterium lepromatosis.
49.) [Lucio's phenomenon in a patient with leprosy on Aruba].
50.) Lucio's phenomenon is a necrotizing panvasculitis: mostly a medium-sized granulomatous
arteritis.
51.) Livedo reticularis in type 2 lepra reaction: A rare presentation.
52.) Is it Lucio Phenomenon or Necrotic Erythema Nodosum Leprosum?
53.) Concomitant lucio phenomenon and erythema nodosum in a leprosy patient: clues for their distinct pathogeneses.
54.) Lucio's phenomenon. Case study of an exceptional response to treatment exclusively with
multibacillary multidrug therapy.
55.) [Lucio-Latapí leprosy and the Lucio phenomenon].
56.) Unusual Presentation of Necrotic Erythema Nodosum Leprosum on Scalp: A Case Report.
57.) Lucio's phenomenon: another case reported in Brazil.
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1.) Lepra type reactions
=============================================================
Source: Mandell, Douglas and Bennett's
Principles and Practice of Infectious Diseases Fourth Edition: 1.995
Lepra Type-1 Reactions (Downgrading and Reversal Reactions)
=========================================
Borderline leprosy patients (BT to BL) may develop before therapy (downgrading reaction) or after the initiation of therapy (reversal reaction) inflammation within previous skin lesions; neuritis; at times, new, multiple, small "satellite" maculopapular skin lesions; and low-grade fever. If neuritis is not treated within the first 24 hours or so, irreversible nerve damage and consequent deformity and muscular dysfunction may result. Reversal reactions are associated with histologic shifts toward the tuberculoid end of the spectrum. Lesions demonstrate increased numbers of CD4+ helper cells, increased levels of interferon-g and IL-2, and an abundance of T cells bearing g- and d-receptors. This pattern is shared with Mitsuda-positive skin tests and does not occur spontaneously otherwise in leprosy.
========================================
Lepra Type-2 Reaction (Erythema Nodosum Leprosum)
========================================
Erythema nodosum leprosum (ENL) is a syndrome affecting nearly half of lepromatous leprosy patients, 90 percent of the time occurring after the initiation of antimicrobial therapy and generally within the first 2 years of treatment. Clinical manifestations include, in order of frequency, painful papules, generally on the extensor surfaces of extremities, which
may pustulate and ulcerate and may appear as recurrent crops; neuritis
(most frequently the ulnar nerve); fever; uveitis; lymphadenitis; orchitis; and glomerulonephritis. Lepromatous leprosy patients should be forewarned of signs and symptoms of ENL, lest their appearance result in loss of confidence with antimicrobial therapy and noncompliance. ENL is histologically an acute vasculitis or paniculitis primarily thought to be secondary to immune complex deposition. It is also thought to be associated with a local increase in cell-mediated immunity: increased numbers of T helper cells and levels of IL-2 and interferon-g, and loss of suppresor T-cell activity. 61
Lucio's Reaction and Nerve Abscesses
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Patients with diffuse lepromatosis may develop shallow, often large polygonal sloughing ulcerations on the lower extremities that heal poorly, are frequently recurrent, and may be generalized. Histopathologically, these lesions appear to be either a variant of ENL or a result of arteriolar infarction. When generalized, Lucio's reaction is frequently fatal, generally a result of secondary bacterial infection and sepsis. Also, leprosy patients, particularly BT or neural leprosy patients, may develop nerve abscesses requiring urgent surgical decompression and drainage.
Therapy of Reactions > ================ Lepra type-1 ================ reactions can be effectively treated only with corticosteroids. We generally initiate therapy with prednisone, 40-60 mg daily. Because relapses commonly occur if steroids are rapidly discontinued, steroids at reduced doses as signs and symptoms allow must be maintained for 2-3 months. Because of the requirement that steroids be maintained for long durations, strict indications are neuritis, lesions that threaten to ulcerate, and lesions that appear on cosmetically important places such as the face.
Lepra type-2 ============= reactions (ENL) can also be effectively treated with corticosteroids, and short durations are often sufficient. If ENL is recurrent, thalidomide in a nightly dose of 100-300 mg is the treatment of choice. In the United States, thalidomide cannot be prescribed to fertile women on an outpatient basis and remains classified as an investigational new drug (IND) with investigators at the G.W. Long Hansen's Disease Center and a number of U.S. Public Health Service-sponsored Regional Ambulatory Hansen's Disease Programs. The mechanism of action of thalidomide for ENL is not fully understood but may be a result of its action to reduce IgM synthesis, 62 retard polymorphonuclear leucocyte migration, 63 and reduce tumor necrosis factor (TNF) levels. 64 Other than resultant birth defects when administered to pregnant women in the first trimester, thalidomide is nontoxic, its only side effects being tranquilization, to which tolerance develops quickly, as well as mild leukopenia, and constipation.
Lucio's Reaction
================
Neither thalidomide nor corticosteroid therapy has proved effective therapy for Lucio's reaction. In severe cases, exchange transfusion may be effective. In general, however, the principals of good wound care and appropriate antibiotics for sepsis are recommended.
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2.) Diffuse Painless Ulcerations
1.) Lepra type reactions
=============================================================
Source: Mandell, Douglas and Bennett's
Principles and Practice of Infectious Diseases Fourth Edition: 1.995
Lepra Type-1 Reactions (Downgrading and Reversal Reactions)
=========================================
Borderline leprosy patients (BT to BL) may develop before therapy (downgrading reaction) or after the initiation of therapy (reversal reaction) inflammation within previous skin lesions; neuritis; at times, new, multiple, small "satellite" maculopapular skin lesions; and low-grade fever. If neuritis is not treated within the first 24 hours or so, irreversible nerve damage and consequent deformity and muscular dysfunction may result. Reversal reactions are associated with histologic shifts toward the tuberculoid end of the spectrum. Lesions demonstrate increased numbers of CD4+ helper cells, increased levels of interferon-g and IL-2, and an abundance of T cells bearing g- and d-receptors. This pattern is shared with Mitsuda-positive skin tests and does not occur spontaneously otherwise in leprosy.
========================================
Lepra Type-2 Reaction (Erythema Nodosum Leprosum)
========================================
Erythema nodosum leprosum (ENL) is a syndrome affecting nearly half of lepromatous leprosy patients, 90 percent of the time occurring after the initiation of antimicrobial therapy and generally within the first 2 years of treatment. Clinical manifestations include, in order of frequency, painful papules, generally on the extensor surfaces of extremities, which
may pustulate and ulcerate and may appear as recurrent crops; neuritis
(most frequently the ulnar nerve); fever; uveitis; lymphadenitis; orchitis; and glomerulonephritis. Lepromatous leprosy patients should be forewarned of signs and symptoms of ENL, lest their appearance result in loss of confidence with antimicrobial therapy and noncompliance. ENL is histologically an acute vasculitis or paniculitis primarily thought to be secondary to immune complex deposition. It is also thought to be associated with a local increase in cell-mediated immunity: increased numbers of T helper cells and levels of IL-2 and interferon-g, and loss of suppresor T-cell activity. 61
Lucio's Reaction and Nerve Abscesses
============================
Patients with diffuse lepromatosis may develop shallow, often large polygonal sloughing ulcerations on the lower extremities that heal poorly, are frequently recurrent, and may be generalized. Histopathologically, these lesions appear to be either a variant of ENL or a result of arteriolar infarction. When generalized, Lucio's reaction is frequently fatal, generally a result of secondary bacterial infection and sepsis. Also, leprosy patients, particularly BT or neural leprosy patients, may develop nerve abscesses requiring urgent surgical decompression and drainage.
Therapy of Reactions > ================ Lepra type-1 ================ reactions can be effectively treated only with corticosteroids. We generally initiate therapy with prednisone, 40-60 mg daily. Because relapses commonly occur if steroids are rapidly discontinued, steroids at reduced doses as signs and symptoms allow must be maintained for 2-3 months. Because of the requirement that steroids be maintained for long durations, strict indications are neuritis, lesions that threaten to ulcerate, and lesions that appear on cosmetically important places such as the face.
Lepra type-2 ============= reactions (ENL) can also be effectively treated with corticosteroids, and short durations are often sufficient. If ENL is recurrent, thalidomide in a nightly dose of 100-300 mg is the treatment of choice. In the United States, thalidomide cannot be prescribed to fertile women on an outpatient basis and remains classified as an investigational new drug (IND) with investigators at the G.W. Long Hansen's Disease Center and a number of U.S. Public Health Service-sponsored Regional Ambulatory Hansen's Disease Programs. The mechanism of action of thalidomide for ENL is not fully understood but may be a result of its action to reduce IgM synthesis, 62 retard polymorphonuclear leucocyte migration, 63 and reduce tumor necrosis factor (TNF) levels. 64 Other than resultant birth defects when administered to pregnant women in the first trimester, thalidomide is nontoxic, its only side effects being tranquilization, to which tolerance develops quickly, as well as mild leukopenia, and constipation.
Lucio's Reaction
================
Neither thalidomide nor corticosteroid therapy has proved effective therapy for Lucio's reaction. In severe cases, exchange transfusion may be effective. In general, however, the principals of good wound care and appropriate antibiotics for sepsis are recommended.
=============================================================
2.) Diffuse Painless Ulcerations
=============================================================
Fernando Gallardo Hernández, MD; Jaime Notario Rosa, MD; Anna Jucglà Serra, MD; Jordi Peyri Rey, MD Ciutat Sanitària i Universitària de Bellvitge, Barcelona, Spain Arch Dermatol, Vol. 135 No. 8, August 1999
REPORT OF A CASE
A 62-year-old man presented with a 3-month history of severe, ulcerating skin lesions over the extremities. Painless violaceous macules, livedo, and occasional bullae gave rise to ulcerations after a few weeks. The lower extremities were predominantly involved, with spontaneous amputation of some necrotic digits. The lesions extended proximally over the buttocks. The patient had a 4- to 5-year history of frequent painless traumatic wounds that healed after 2 to 3 weeks, leaving atrophic scars.
The patient also noted progressive thickening of the skin, with loss of body hair, especially the eyebrows. Physical examination revealed a chronically ill man with waxy diffuse skin infiltration and a total loss of body hair. Numerous angular ulcerations were located over the extremities (Figure 1) and surrounded by a livedoid pattern. The findings of the rest of his examination were unremarkable except for a sensitive polyneuropathy. Laboratory evaluation showed the following abnormal values: hemoglobin, 90 g/L; mean corpuscular volume, 67 pg; erythrocyte sedimentation rate, 120 mm/h; and serum albumin, 16 g/L. The serum concentration of -globulin was increased in a polyclonal pattern. A skin biopsy specimen was obtained (Figure 2), and Ziehl-Neelsen staining was performed (Figure 3). (see the attach file)
Diagnosis: Lucio phenomenon.
HISTOPATHOLOGIC FINDINGS AND CLINICAL COURSE
A punch biopsy specimen from the border of an ulcer revealed a patchy infiltrate of foamy histiocytes and scattered lymphocytes in the dermis and subcutaneous fat, tending to be clustered around blood vessels. Large number of acid-fast bacilli were present in the Ziehl-Neelsen stain of the specimen. Foamy histiocytes and acid-fast bacilli were also noted in the wall of the medium-sized arteries, with narrowing of the vessel lumen. No nuclear dust was observed. Slit-skin smears showed single and clustered acid-fast bacilli.
Multidrug therapy was initiated with 100 mg/d of oral dapsone, 50 mg/d of clofazimine, 600 mg/d of rifampin, and a single 300-mg dose of clofazimine per month, with good response to date. Prednisone (40 mg) was also prescribed at decreasing doses for Lucio phenomenon and has been required for months at low doses. Our patient's ulcers healed with irregular atrophic scars in 10 to 12 weeks.
DISCUSSION;
Our patient manifested the clinical and histopathologic features of a diffuse, lepromatous leprosy with skin ulcerations characteristic of Lucio phenomenon, a severe, necrotizing reaction described by Lucio and Alvarado1 in 1852. The reactional states in leprosy are characterized by acute inflammatory changes, and they occur more commonly toward the borderline and lepromatous end of the clinical spectrum. Lucio phenomenon is an infrequent reaction in leprosy. It has usually been described in diffuse lepromatous leprosy, so-called Lucio leprosy, which is unique because of the absence of cutaneous nodules. This type of leprosy is almost exclusively seen in Mexico and Central America. Lucio phenomenon is a cutaneous necrotizing reaction that most commonly involves the extremities but may also affect the buttocks and trunk. The acute constitutional symptoms of Lucio phenomenon are variable and, occasionally, may be fatal.2, 3 It is believed to be an immune complex–mediated disease like the Arthus phenomenon and is included as a type 2 reaction in leprosy.4, 5 Lucio phenomenon shows histopathologic findings of leukocytoclastic vasculitis along with a superficial and deep mixed infiltrate of foamy histiocytes in a perineural and perivascular distribution. In some reported cases, the vascular damage may have been the result of direct invasion of Mycobacterium leprae, with endothelial cell proliferation, thrombosis, and tissue necrosis.6
The treatment of Lucio phenomenon is the same as that for other type 2 reactions in leprosy. Local care and supportive therapy are important to prevent secondary infections and loss of proteins. Prednisone therapy, which is initially administered at dosages of 20 to 60 mg/d and then tapered, may be required for weeks to months. Thalidomide (400 mg/d with reduction to maintenance doses of 100 mg/d) may be prescribed as an alternative to prednisone. Clofazimine administered at a dosage of 300 mg/d has a useful anti-inflammatory effect. It is not necessary to discontinue multidrug therapy for leprosy.
REFERENCES
1. Lucio R, Alvarado Y, Latapi F, Cited by, Zamora AC, Cited by. The spotted leprosy of Lucio. Int J Lepr. 1948;16:421-430.
2. Pursley TV, Jacobson RR. Lucio's phenomenon. Arch Dermatol. 1980;116: 201-204. MEDLINE
3. Bernadat JP, Faucher JF, Huerre M. Lèpre lépromateuse diffuse révélée par une vasculite cutanée: le phenomene de Lucio. Ann Dermatol Venereol. 1996;123:21-23. MEDLINE
4. Touma DJ, Phillips TJ, Kurban AK, Goldberg L. Recurrent rapidly progressive infiltrated plaques and bullae. Arch Dermatol. 1996;132:1432-1434. MEDLINE
5. Murphy GF, Sánchez NP, Flynn TC, Sánchez JL, Mihm MC, Soter NA. Erythema nodosum leprosum. J Am Acad Dermatol. 1986;14:59-69. MEDLINE
6. Rea TH, Levan NE. Lucio's phenomenon and diffuse nonnodular lepromatous
leprosy. Arch Dermatol. 1978;114:1023-1028. MEDLINE
=============================================================
3.) [Lucio-Latapi leprosy and the Lucio phenomenon]
============================================================
ARTICLE SOURCE: Acta Leprol (Switzerland), Jul-Sep 1983, 1(3) p115-32
AUTHOR(S): Saul A; Novales J
PUBLICATION TYPE: JOURNAL ARTICLE; REVIEW (18 references)
ABSTRACT:
The Lucio-Latapi's leprosy or diffuse lepromatous leprosy is a clinical variety of lepromatous leprosy first described by Lucio and Alvarado in 1852 and reidentified by Latapi in 1936. It is frequent in Mexico (23%) and in Costa Rica and very rare in other countries. It is characterized by a diffuse infiltration of all the skin which never is transformed into nodule, by a complete alopecia of eyebrows and eyelashes and body hair, by anhydrotic and dysesthesic zones of the skin and by a peculiar type of lepra reaction named Lucio's phenomenon or necrotic erythema which is a vascularitis of vessels especially of the dermohypodermic union and of the hypodermis. Clinically this vascularitis is represented by well-shaped erythematous spots, later becoming necrotic with scabs, ulcerations and scars. Three points of confusion are stressed: the differences between nodules and nudosities, Lucio's leprosy and Lucio's phenomenon and necrotic erythema and necrotic erythema nodosum leprosum. The differences between the pure and primitive form of Lucio's leprosy and the secondary one is also discussed such as the laboratory findings, histopathological data, pronostic and treatment. Lucio's leprosy is considered the most anergic one of the all immunological spectrum of leprosy.
=============================================================
4.) [Lucio's leprosy].
=============================================================
Med Cutan Ibero Lat Am 1982;10(1):41-6 Related Articles, Books, LinkOut
Gibert E, Cubria JL, Gratacos R, Castro J, Monfort J, Castel T, Lecha M
A case of diffuse lepromatous leprosy with lepra reaction type II-Lucio's phenomenon-in a 24 years old male patient is reported. The histological examination of the necrotic lesions and of the apparently normal skin showed the presence of dense perivascular and perianexial lymphohystiocitic infiltrates with great quantities of bacilli. The first biopsy did not show a picture a leuccocytoclastic vasculitis but only areas of necrosis. The immunofluorescence studies revealed on direct examination complement deposits on vessel walls. The complement levels in blood were lowered and circulating inmunecomplexes were also detected. These data confirm the opinion that Lucio's phenomenon is caused by circulating inmunecomplexes fixed on dermal vessel walls causing skin necrotic lesions.
=============================================================
5.) [22 years of leprosy: histopathology]
=============================================================
AU: Dionisio-de-Cabalier-ME; Perez-HJ
AD: Ia. Catedra de Patologia, Facultad de Ciencias Medicas, Hospital Nac,
de Clinicas, U.N.C.
SO: Rev-Fac-Cien-Med-Univ-Nac-Cordoba. 1995; 53(1): 17-21 AB: In the present study, the frequency of histopathological reports of leprosy carried out in the last 22 years at the Fst Chair of Pathology (Medical School, U. N.C) was determined. Our findings on the frequency of pure forms of leprosy agree with those reported by the O.M.S. On the contrary, that was not the case with respect to reactive forms, since the phenomenon of Lucio and Alvarado was more frequent in endemic zones.
=============================================================
6.) Lucio's phenomenon.
============================================================= ARTICLE SOURCE: Arch Dermatol (United States), Feb 1980, 116(2) p201-4
AUTHOR(S): Pursley TV; Jacobson RR
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
A 38-year-old woman had diffuse, nonnodular, lepromatous
leprosy and Lucio's phenomenon. Most cases of Lucio's phenomenon have been reported to have a leukocytoclastic vasculitis as the underlying pathologic abnormality. In this patient, however, the histologic picture of an early lesion of Lucio's phenomenon showed a milk, mononuclear cell infiltration, endothelial swelling, vascular thrombosis, and ischemic necrosis. Lepra bacilli were abundant around nerves and blood vessels, and many were noted in vascular walls and endothelium. Our findings raise the possibility that some cases of Lucio's phenomenon may be caused by vascular damage due to direct invasion of Mycobacterium leprae and not necessarily by leukocytoclastic vasculitis.
=============================================================
7.) Lucio's phenomenon and diffuse nonnodular lepromatous leprosy.
=============================================================
ARTICLE SOURCE: Arch Dermatol (United States), Jul 1978, 114(7) p1023-8
AUTHOR(S): Rea TH; Levan NE
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
The records of ten patients with Lucio's phenomenon showed clinical and histopathological changes similar to those described by others. Lucio's phenomenon is a syndrome distinct from erythema nodosum leprosum as indicated by an absence of fever, leukocytosis and tenderness, a failure to respond to thalidomide, and a restriction to patients with diffuse nonnodular lepromatous leprosy. Lymphopenia associated with splenomegaly in three patients and glomerulonephritis in one patient were unexpected findings of unknown relevance.
=============================================================
8.) Lucio's phenomenon: a comparative histological study.
Fernando Gallardo Hernández, MD; Jaime Notario Rosa, MD; Anna Jucglà Serra, MD; Jordi Peyri Rey, MD Ciutat Sanitària i Universitària de Bellvitge, Barcelona, Spain Arch Dermatol, Vol. 135 No. 8, August 1999
REPORT OF A CASE
A 62-year-old man presented with a 3-month history of severe, ulcerating skin lesions over the extremities. Painless violaceous macules, livedo, and occasional bullae gave rise to ulcerations after a few weeks. The lower extremities were predominantly involved, with spontaneous amputation of some necrotic digits. The lesions extended proximally over the buttocks. The patient had a 4- to 5-year history of frequent painless traumatic wounds that healed after 2 to 3 weeks, leaving atrophic scars.
The patient also noted progressive thickening of the skin, with loss of body hair, especially the eyebrows. Physical examination revealed a chronically ill man with waxy diffuse skin infiltration and a total loss of body hair. Numerous angular ulcerations were located over the extremities (Figure 1) and surrounded by a livedoid pattern. The findings of the rest of his examination were unremarkable except for a sensitive polyneuropathy. Laboratory evaluation showed the following abnormal values: hemoglobin, 90 g/L; mean corpuscular volume, 67 pg; erythrocyte sedimentation rate, 120 mm/h; and serum albumin, 16 g/L. The serum concentration of -globulin was increased in a polyclonal pattern. A skin biopsy specimen was obtained (Figure 2), and Ziehl-Neelsen staining was performed (Figure 3). (see the attach file)
Diagnosis: Lucio phenomenon.
HISTOPATHOLOGIC FINDINGS AND CLINICAL COURSE
A punch biopsy specimen from the border of an ulcer revealed a patchy infiltrate of foamy histiocytes and scattered lymphocytes in the dermis and subcutaneous fat, tending to be clustered around blood vessels. Large number of acid-fast bacilli were present in the Ziehl-Neelsen stain of the specimen. Foamy histiocytes and acid-fast bacilli were also noted in the wall of the medium-sized arteries, with narrowing of the vessel lumen. No nuclear dust was observed. Slit-skin smears showed single and clustered acid-fast bacilli.
Multidrug therapy was initiated with 100 mg/d of oral dapsone, 50 mg/d of clofazimine, 600 mg/d of rifampin, and a single 300-mg dose of clofazimine per month, with good response to date. Prednisone (40 mg) was also prescribed at decreasing doses for Lucio phenomenon and has been required for months at low doses. Our patient's ulcers healed with irregular atrophic scars in 10 to 12 weeks.
DISCUSSION;
Our patient manifested the clinical and histopathologic features of a diffuse, lepromatous leprosy with skin ulcerations characteristic of Lucio phenomenon, a severe, necrotizing reaction described by Lucio and Alvarado1 in 1852. The reactional states in leprosy are characterized by acute inflammatory changes, and they occur more commonly toward the borderline and lepromatous end of the clinical spectrum. Lucio phenomenon is an infrequent reaction in leprosy. It has usually been described in diffuse lepromatous leprosy, so-called Lucio leprosy, which is unique because of the absence of cutaneous nodules. This type of leprosy is almost exclusively seen in Mexico and Central America. Lucio phenomenon is a cutaneous necrotizing reaction that most commonly involves the extremities but may also affect the buttocks and trunk. The acute constitutional symptoms of Lucio phenomenon are variable and, occasionally, may be fatal.2, 3 It is believed to be an immune complex–mediated disease like the Arthus phenomenon and is included as a type 2 reaction in leprosy.4, 5 Lucio phenomenon shows histopathologic findings of leukocytoclastic vasculitis along with a superficial and deep mixed infiltrate of foamy histiocytes in a perineural and perivascular distribution. In some reported cases, the vascular damage may have been the result of direct invasion of Mycobacterium leprae, with endothelial cell proliferation, thrombosis, and tissue necrosis.6
The treatment of Lucio phenomenon is the same as that for other type 2 reactions in leprosy. Local care and supportive therapy are important to prevent secondary infections and loss of proteins. Prednisone therapy, which is initially administered at dosages of 20 to 60 mg/d and then tapered, may be required for weeks to months. Thalidomide (400 mg/d with reduction to maintenance doses of 100 mg/d) may be prescribed as an alternative to prednisone. Clofazimine administered at a dosage of 300 mg/d has a useful anti-inflammatory effect. It is not necessary to discontinue multidrug therapy for leprosy.
REFERENCES
1. Lucio R, Alvarado Y, Latapi F, Cited by, Zamora AC, Cited by. The spotted leprosy of Lucio. Int J Lepr. 1948;16:421-430.
2. Pursley TV, Jacobson RR. Lucio's phenomenon. Arch Dermatol. 1980;116: 201-204. MEDLINE
3. Bernadat JP, Faucher JF, Huerre M. Lèpre lépromateuse diffuse révélée par une vasculite cutanée: le phenomene de Lucio. Ann Dermatol Venereol. 1996;123:21-23. MEDLINE
4. Touma DJ, Phillips TJ, Kurban AK, Goldberg L. Recurrent rapidly progressive infiltrated plaques and bullae. Arch Dermatol. 1996;132:1432-1434. MEDLINE
5. Murphy GF, Sánchez NP, Flynn TC, Sánchez JL, Mihm MC, Soter NA. Erythema nodosum leprosum. J Am Acad Dermatol. 1986;14:59-69. MEDLINE
6. Rea TH, Levan NE. Lucio's phenomenon and diffuse nonnodular lepromatous
leprosy. Arch Dermatol. 1978;114:1023-1028. MEDLINE
=============================================================
3.) [Lucio-Latapi leprosy and the Lucio phenomenon]
============================================================
ARTICLE SOURCE: Acta Leprol (Switzerland), Jul-Sep 1983, 1(3) p115-32
AUTHOR(S): Saul A; Novales J
PUBLICATION TYPE: JOURNAL ARTICLE; REVIEW (18 references)
ABSTRACT:
The Lucio-Latapi's leprosy or diffuse lepromatous leprosy is a clinical variety of lepromatous leprosy first described by Lucio and Alvarado in 1852 and reidentified by Latapi in 1936. It is frequent in Mexico (23%) and in Costa Rica and very rare in other countries. It is characterized by a diffuse infiltration of all the skin which never is transformed into nodule, by a complete alopecia of eyebrows and eyelashes and body hair, by anhydrotic and dysesthesic zones of the skin and by a peculiar type of lepra reaction named Lucio's phenomenon or necrotic erythema which is a vascularitis of vessels especially of the dermohypodermic union and of the hypodermis. Clinically this vascularitis is represented by well-shaped erythematous spots, later becoming necrotic with scabs, ulcerations and scars. Three points of confusion are stressed: the differences between nodules and nudosities, Lucio's leprosy and Lucio's phenomenon and necrotic erythema and necrotic erythema nodosum leprosum. The differences between the pure and primitive form of Lucio's leprosy and the secondary one is also discussed such as the laboratory findings, histopathological data, pronostic and treatment. Lucio's leprosy is considered the most anergic one of the all immunological spectrum of leprosy.
=============================================================
4.) [Lucio's leprosy].
=============================================================
Med Cutan Ibero Lat Am 1982;10(1):41-6 Related Articles, Books, LinkOut
Gibert E, Cubria JL, Gratacos R, Castro J, Monfort J, Castel T, Lecha M
A case of diffuse lepromatous leprosy with lepra reaction type II-Lucio's phenomenon-in a 24 years old male patient is reported. The histological examination of the necrotic lesions and of the apparently normal skin showed the presence of dense perivascular and perianexial lymphohystiocitic infiltrates with great quantities of bacilli. The first biopsy did not show a picture a leuccocytoclastic vasculitis but only areas of necrosis. The immunofluorescence studies revealed on direct examination complement deposits on vessel walls. The complement levels in blood were lowered and circulating inmunecomplexes were also detected. These data confirm the opinion that Lucio's phenomenon is caused by circulating inmunecomplexes fixed on dermal vessel walls causing skin necrotic lesions.
=============================================================
5.) [22 years of leprosy: histopathology]
=============================================================
AU: Dionisio-de-Cabalier-ME; Perez-HJ
AD: Ia. Catedra de Patologia, Facultad de Ciencias Medicas, Hospital Nac,
de Clinicas, U.N.C.
SO: Rev-Fac-Cien-Med-Univ-Nac-Cordoba. 1995; 53(1): 17-21 AB: In the present study, the frequency of histopathological reports of leprosy carried out in the last 22 years at the Fst Chair of Pathology (Medical School, U. N.C) was determined. Our findings on the frequency of pure forms of leprosy agree with those reported by the O.M.S. On the contrary, that was not the case with respect to reactive forms, since the phenomenon of Lucio and Alvarado was more frequent in endemic zones.
=============================================================
6.) Lucio's phenomenon.
============================================================= ARTICLE SOURCE: Arch Dermatol (United States), Feb 1980, 116(2) p201-4
AUTHOR(S): Pursley TV; Jacobson RR
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
A 38-year-old woman had diffuse, nonnodular, lepromatous
leprosy and Lucio's phenomenon. Most cases of Lucio's phenomenon have been reported to have a leukocytoclastic vasculitis as the underlying pathologic abnormality. In this patient, however, the histologic picture of an early lesion of Lucio's phenomenon showed a milk, mononuclear cell infiltration, endothelial swelling, vascular thrombosis, and ischemic necrosis. Lepra bacilli were abundant around nerves and blood vessels, and many were noted in vascular walls and endothelium. Our findings raise the possibility that some cases of Lucio's phenomenon may be caused by vascular damage due to direct invasion of Mycobacterium leprae and not necessarily by leukocytoclastic vasculitis.
=============================================================
7.) Lucio's phenomenon and diffuse nonnodular lepromatous leprosy.
=============================================================
ARTICLE SOURCE: Arch Dermatol (United States), Jul 1978, 114(7) p1023-8
AUTHOR(S): Rea TH; Levan NE
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
The records of ten patients with Lucio's phenomenon showed clinical and histopathological changes similar to those described by others. Lucio's phenomenon is a syndrome distinct from erythema nodosum leprosum as indicated by an absence of fever, leukocytosis and tenderness, a failure to respond to thalidomide, and a restriction to patients with diffuse nonnodular lepromatous leprosy. Lymphopenia associated with splenomegaly in three patients and glomerulonephritis in one patient were unexpected findings of unknown relevance.
=============================================================
8.) Lucio's phenomenon: a comparative histological study.
=============================================================
ARTICLE SOURCE: Int J Lepr Other Mycobact Dis (United States), Jun 1979, 47(2) p161-6
AUTHOR(S): Rea TH; Ridley DS
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
To study further the pathogenesis of Lucio's phenomenon, we have made a comparative histological study of 11 patients with Lucio's phenomenon and 12 with ENL. Confirming the findings of others, Lucio's reaction could be distinguished from ENL by epidermal necrosis and by necrotizing vasculitis manifesting necrosis in the walls of superficial vessels and severe, focal endothelial proliferation of mid-dermal vessels. Furthermore, in Lucio's phenomenon large numbers of AFB were found in evidently normal and in swollen or proliferating endothelial cells. We hypothesize that patients with Lucio's phenomenon have an exceptionally deficient defense mechanism, allowing unrestricted proliferation of AFB in endothelial cells, facilitating contact between bacterial antigen and circulating antibody and leading to infarction; also, this nadir of resistance allows unimpeded dissemination of AFB, accounting for the clinical features of diffuse non-nodular leprosy. Thus, an explanation is offered for the restriction of Lucio's phenomenon to patients with diffuse non-nodular lepromatous leprosy.
=============================================================
9.) [Diffuse lepromatous leprosy disclosed by cutaneous vasculitis. The Lucio phenomenon].
=============================================================
Ann Dermatol Venereol 1996;123(1):21-3
Bernadat JP, Faucher JF, Huerre M
Clinique Paofai, Papeete, Tahiti, Polynesie francaise.
INTRODUCTION:
Lucio's phenomenon, also called necrotizing erythema, is a rare acute manifestation which sometimes introduces diffuse lepromatous leprosy, almost exclusively in Central American populations.
CASE REPORT:
A 76-year-old polynesian man of chinese ethnic origin had necrotizing erythema for several months before development of Lucio's leprosy. The patient had necrotizing lesions of the lower limbs with large polygonal scars and poor general health status. Diagnosis was based on the discovery of acid-fast bacilli at the pathology examination of skin biopsies. The necrotizing zones appeared as cutaneous vasculitis with angiogenesis of the superficial dermis and presence of Hansen bacilli within the endothelium.
DISCUSSION:
This case of diffuse lepromatous leprosy, the first reported in the South Pacific, emphasizes the polymorphism of leprosy and the importance of recognizing rare clinical forms, especially in the tropics. Anti-Hansen drugs are effective.
=============================================================
10.) Immunologic aspects of leprosy as related to leucocytic isoantibodies and platelet aggregating factors.
=============================================================
ARTICLE SOURCE: Int J Lepr Other Mycobact Dis (United States), Jul-Sep 1975, 43(3) p239-48
AUTHOR(S): Saha K; Dutta RN; Mittal MM
PUBLICATION TYPE:
JOURNAL ARTICLE
ABSTRACT:
The incidences of various iso- and autoantibodies in a random population of 112 unselected leprosy patients is presented. Low titers of leucocytic isoantibodies and platelet aggregating factor were detected in the sera of a variable number of such patients. The leucoisoagglutinins were found in 8% of the sera of tuberculoid as well as lepromatous leprosy patients, whereas the leucoisocytotoxins were detected in a larger percentage of the lepromatous (40%) as well as tuberculoid (28%) cases. The platelet aggregating factors (PAF) were positive in 51.2% and 45% of lepromatous and tuberculoid cases respectively. Of the 21 positive sera for PAF, the antiplatelet factor by antihuman globulin consumption test could be demonstrated only in 66.6% and 50% of lepromatous and tuberculoid sera respectively.
To study the frequencies of these newly detected antibodies or antibody-like factor and to compare their occurrences with other well-documented autoantibodies present in the sera of leprosy patients: cryoglobulins, antinucleoprotein antibody and thyroglobulin autoprecipitin were also studied in the sera of the same population of leprosy patients. It has been observed that the simultaneous occurrence of all these auto- and isoantibodies in the serum of one patient is a rare phenomenon. Leucocytic and platelet counts of these patients having antibodies against leucocytes and platelets were found to be within normal limits.
Accordingly, it is suggested that the low levels of antileucocyte antibody and antiplatelet factor are probably harmless to the hosts. On the other hand, it is postulated that these antibodies may act as enhancing factors by being specifically adsorbed on the lymphoid cells, thus rendering them unresponsive to mitogenic stimulus in vitro. From these studies it seems that leprosy, especially the lepromatous type, is associated with some of the serological features suggestive of an autoimmune aberration.
=============================================================
11.) The role of protein malnutrition in the pathogenesis of ulcerative "Lazarine" leprosy.
ARTICLE SOURCE: Int J Lepr Other Mycobact Dis (United States), Jun 1979, 47(2) p161-6
AUTHOR(S): Rea TH; Ridley DS
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
To study further the pathogenesis of Lucio's phenomenon, we have made a comparative histological study of 11 patients with Lucio's phenomenon and 12 with ENL. Confirming the findings of others, Lucio's reaction could be distinguished from ENL by epidermal necrosis and by necrotizing vasculitis manifesting necrosis in the walls of superficial vessels and severe, focal endothelial proliferation of mid-dermal vessels. Furthermore, in Lucio's phenomenon large numbers of AFB were found in evidently normal and in swollen or proliferating endothelial cells. We hypothesize that patients with Lucio's phenomenon have an exceptionally deficient defense mechanism, allowing unrestricted proliferation of AFB in endothelial cells, facilitating contact between bacterial antigen and circulating antibody and leading to infarction; also, this nadir of resistance allows unimpeded dissemination of AFB, accounting for the clinical features of diffuse non-nodular leprosy. Thus, an explanation is offered for the restriction of Lucio's phenomenon to patients with diffuse non-nodular lepromatous leprosy.
=============================================================
9.) [Diffuse lepromatous leprosy disclosed by cutaneous vasculitis. The Lucio phenomenon].
=============================================================
Ann Dermatol Venereol 1996;123(1):21-3
Bernadat JP, Faucher JF, Huerre M
Clinique Paofai, Papeete, Tahiti, Polynesie francaise.
INTRODUCTION:
Lucio's phenomenon, also called necrotizing erythema, is a rare acute manifestation which sometimes introduces diffuse lepromatous leprosy, almost exclusively in Central American populations.
CASE REPORT:
A 76-year-old polynesian man of chinese ethnic origin had necrotizing erythema for several months before development of Lucio's leprosy. The patient had necrotizing lesions of the lower limbs with large polygonal scars and poor general health status. Diagnosis was based on the discovery of acid-fast bacilli at the pathology examination of skin biopsies. The necrotizing zones appeared as cutaneous vasculitis with angiogenesis of the superficial dermis and presence of Hansen bacilli within the endothelium.
DISCUSSION:
This case of diffuse lepromatous leprosy, the first reported in the South Pacific, emphasizes the polymorphism of leprosy and the importance of recognizing rare clinical forms, especially in the tropics. Anti-Hansen drugs are effective.
=============================================================
10.) Immunologic aspects of leprosy as related to leucocytic isoantibodies and platelet aggregating factors.
=============================================================
ARTICLE SOURCE: Int J Lepr Other Mycobact Dis (United States), Jul-Sep 1975, 43(3) p239-48
AUTHOR(S): Saha K; Dutta RN; Mittal MM
PUBLICATION TYPE:
JOURNAL ARTICLE
ABSTRACT:
The incidences of various iso- and autoantibodies in a random population of 112 unselected leprosy patients is presented. Low titers of leucocytic isoantibodies and platelet aggregating factor were detected in the sera of a variable number of such patients. The leucoisoagglutinins were found in 8% of the sera of tuberculoid as well as lepromatous leprosy patients, whereas the leucoisocytotoxins were detected in a larger percentage of the lepromatous (40%) as well as tuberculoid (28%) cases. The platelet aggregating factors (PAF) were positive in 51.2% and 45% of lepromatous and tuberculoid cases respectively. Of the 21 positive sera for PAF, the antiplatelet factor by antihuman globulin consumption test could be demonstrated only in 66.6% and 50% of lepromatous and tuberculoid sera respectively.
To study the frequencies of these newly detected antibodies or antibody-like factor and to compare their occurrences with other well-documented autoantibodies present in the sera of leprosy patients: cryoglobulins, antinucleoprotein antibody and thyroglobulin autoprecipitin were also studied in the sera of the same population of leprosy patients. It has been observed that the simultaneous occurrence of all these auto- and isoantibodies in the serum of one patient is a rare phenomenon. Leucocytic and platelet counts of these patients having antibodies against leucocytes and platelets were found to be within normal limits.
Accordingly, it is suggested that the low levels of antileucocyte antibody and antiplatelet factor are probably harmless to the hosts. On the other hand, it is postulated that these antibodies may act as enhancing factors by being specifically adsorbed on the lymphoid cells, thus rendering them unresponsive to mitogenic stimulus in vitro. From these studies it seems that leprosy, especially the lepromatous type, is associated with some of the serological features suggestive of an autoimmune aberration.
=============================================================
11.) The role of protein malnutrition in the pathogenesis of ulcerative "Lazarine" leprosy.
=============================================================
ARTICLE SOURCE: Int J Lepr Other Mycobact Dis (United States), Jul-Sep 1976, 44(3) p346-58
AUTHOR(S): Skinsnes LK; Higa LH
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
1. Clinical and necropsy observations in lepromatous leprosy associated with severe emaciation and accompanying hypoproteinemia suggest that protein deprivation may be of pathogenic significance in the ulcerative phenomenon that is designated "Lazarine leprosy".
2. An experimental utilizing Wiersung rats infected with Mycobacterium lepraemurium and maintained on a protein-free diet was developed for the purpose of studying the effect of protein starvation on the course of chronic mycobacterial disease similar to lepromatous leprosy with respect to pathogen and host inflammatory response.
3. It was possible to maintain the experimental animals on a protein-free diet for up to 18 weeks of concomitant M. lepraemurium infection. This was long enough for the infection to disseminate to a degree that was evident in control animals only several weeks later.
4. The protein-deprived animals showed decreased inflammatory response to the pathogen, presented more rapid dissemination of the infection and harbored more bacilli per macrophage than did animals similarly infected but maintained on a protein adequate diet. This indicates impairment of native cellular immunity by protein deprivation through decrease in ability of macrophages to inhibit bacillary multiplication.
5. There was no evidence of impairment of macrophage ability to phagocytose the pathogens.
6. Morphologically the increased dissemination of pathogens and decrease in inflammatory response was similar to the increase in number and extent of visceral lesions seen in Lazarine leprosy. Decreased ability to dispose of the infecting bacilli was similar in the two models, human and animal. The animal model does not, as does lepromatous leprosy, involve the skin in the infection. Hence comparable ulcerative phenomena were not replicated in the animals.
7. It is suggested that Lazarine leprosy may result from enhanced lepromatous leprous infection occurring as a result of protein malnutrition. The pathogenic mechanism appears to be impairment of cellular immunity probably enhanced by concomitant impairment of humoral antibody immunity resulting also in decreased resistance to pyogenic and other secondary pathogens. The tissue edema attendant on decreased serum osmotic pressure due to lowering of the serum protein fractions enhances the probability of ulceration.
=============================================================
12.) Dermal ultrastructure in leprosy. =============================================================
ARTICLE SOURCE: Arch Pathol Lab Med (United States), May 1984, 108(5)
p383-6
AUTHOR(S): Van Hale HM; Turkel SB; Rea TH
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT: We studied the ultrastructure of the dermal inflammatory
response in 18 patients with leprosy. Biopsy specimens from 14 lepromatous patients, including four with Lucio's phenomenon and four with erythema nodosum leprosum, were compared with biopsy specimens from one borderline lepromatous and three borderline tuberculoid patients. In all, the dermal infiltrate consisted of macrophages, lymphocytes, and mast cells.
This infiltrate was predominantly perivascular, and chronic reactive changes were found in the small dermal vessels. The macrophages contained phagocytized organisms within membrane-bound vacuoles and a wide variety of lysosomal residual dense bodies. Intraendothelial organisms were occasionally seen, especially in biopsy specimens from the patients with Lucio's phenomenon. The greatest number of mast cells were also seen in the infiltrate in those cases. The frequent close association of macrophages with lymphocytes and mast cells suggests an interrelationship between these cells that appears typical of the host response to leprosy.
=============================================================
13.) Lepromatous and tuberculoid leprosy: clinical presentation and cytokine responses.
=============================================================
Ochoa MT; Valderrama L; Ochoa A; Zea A; Escobar CE; Moreno LH; Falabella Dermatology Service, Universidad del Valle, Cali, Colombia.
Int J Dermatol (UNITED STATES) Nov 1996 35 (11) p786-90
OBJECTIVE:
This study analyzes the major clinical characteristics of patients with active leprosy in relation to the in vitro immune response to the T-lymphocyte activator anti-CD3.
METHODS:
Thirty-eight patients with an established diagnosis of leprosy were classified according to the Ridley and Jopling table. Peripheral blood mononuclear cells from both lepromatous leprosy (LL) and tuberculoid leprosy (TL) patients and healthy controls were used to evaluate lymphocyte proliferation; immunoenzymatic assays were used to evaluate cytokine production (IL-1, IL-2, IL-4, IL-6, IL-10, IFN-gamma).
RESULTS:
Peripheral blood mononuclear cells from both LL and TL patients displayed blastogenic responses to anti-CD3. The cytokines IL-1 beta, IL-6, IL-10, and IFN-gamma were detected in culture supernatants. Endogenous production of IL-1 beta was significantly higher in cell cultures from patients with the lepromatous form of the disease compared to those with tuberculoid leprosy. Production of IL-6 in response to anti-CD3 was observed in a significantly higher proportion of LL than TL patients (P = 0.0025). Gamma-interferon production did not differ between TL and LL, but a direct correlation was observed between time of multidrug treatment and IFN production in vitro (P = 0.016). Interleukin-10 was detected in culture supernatants of lymphocytes activated by anti-CD3 from both patient groups, but not from healthy controls.
CONCLUSIONS:
The findings of this study suggest that patients with the two distinct forms of leprosy are capable of responding to a polyclonal T-lymphocyte stimulus such as anti-CD3 and provide evidence suggestive of alterations in the immune responses mediated by cytokines that may contribute to the spectrum of disease and response to treatment.
=============================================================
14.) Leprosy (Hansen's disease) in South Dakota.
=============================================================
S D J Med 1996 Jun;49(6):185-7 Related Articles, Books, LinkOut
Burrish G, Hartmann A, Lockwood W
Department of Dermatology, Central Plains Clinic, Sioux Falls, SD, USA.
Worldwide Hansen's disease is an important and relatively common disease, but is still very rare in South Dakota. Two patients are described to help demonstrate the wide variety of clinical manifestations associated with Hansen's disease. Since the clinical appearance of Hansen's disease is highly variable, the following six forms of Hansen's disease are described: Indeterminate, tuberculoid (TT), borderline tuberculoid (BT), borderline (BB), borderline lepromatous (BL), and lepromatous leprosy (LL). In addition, three well-recognized reactional forms of leprosy are also described: Type 1 (lepra reaction), type 2 (erythema nodosum leprosum), and type 3 (Lucio's phenomenon). While the disease affects primarily the skin and nerves, health care providers of all disciplines should remain alert for this disease which can present with a high degree of clinical variability.
=============================================================
15.) [Virchowian Hansen's disease, Lucio's phenomenon, cryptococcosis].
=============================================================
Hansenol Int 1988 Dec;13(2):47-56 Related Articles, Books, LinkOut
[Article in Portugese]
A 75 years old white male, for 3 years on treatment for virchowian hanseniasis, was admitted with active HD lesions, infiltration on the base of right lung, leg ulcer and malaise. After two days he developed purpura and hemorrhagic blisters in the limbs. The biopsy of these lesions revealed Lucio phenomenon. The patient worsened with mental confusion, psychomotor agitation and anisocoric pupils. In the 18th day of internation the patient died. Necropsy revealed virchowian infiltration plenty of bacilli in the skin and viscera as well as tuberculoid granuloma with acid-fast bacilli in the liver, spleen and bone marrow. These findings lead us to review the patient's classification from virchowian to borderline. In the lungs, leptomeninge, renal papile, prostate and thyroid it was found loose tuberculoid granuloma with a great amount of fungi surrounded by a gelly halo resembling Criptococcus neoformans. These findings and the onset of Lucio phenomenon are discussed in a patient that has been treated for 3 years and still having several virchowian lesions and a great amount of acid-fast bacilli.
=============================================================
16.) Erythema nodosum leprosum in Singapore.
ARTICLE SOURCE: Int J Lepr Other Mycobact Dis (United States), Jul-Sep 1976, 44(3) p346-58
AUTHOR(S): Skinsnes LK; Higa LH
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT:
1. Clinical and necropsy observations in lepromatous leprosy associated with severe emaciation and accompanying hypoproteinemia suggest that protein deprivation may be of pathogenic significance in the ulcerative phenomenon that is designated "Lazarine leprosy".
2. An experimental utilizing Wiersung rats infected with Mycobacterium lepraemurium and maintained on a protein-free diet was developed for the purpose of studying the effect of protein starvation on the course of chronic mycobacterial disease similar to lepromatous leprosy with respect to pathogen and host inflammatory response.
3. It was possible to maintain the experimental animals on a protein-free diet for up to 18 weeks of concomitant M. lepraemurium infection. This was long enough for the infection to disseminate to a degree that was evident in control animals only several weeks later.
4. The protein-deprived animals showed decreased inflammatory response to the pathogen, presented more rapid dissemination of the infection and harbored more bacilli per macrophage than did animals similarly infected but maintained on a protein adequate diet. This indicates impairment of native cellular immunity by protein deprivation through decrease in ability of macrophages to inhibit bacillary multiplication.
5. There was no evidence of impairment of macrophage ability to phagocytose the pathogens.
6. Morphologically the increased dissemination of pathogens and decrease in inflammatory response was similar to the increase in number and extent of visceral lesions seen in Lazarine leprosy. Decreased ability to dispose of the infecting bacilli was similar in the two models, human and animal. The animal model does not, as does lepromatous leprosy, involve the skin in the infection. Hence comparable ulcerative phenomena were not replicated in the animals.
7. It is suggested that Lazarine leprosy may result from enhanced lepromatous leprous infection occurring as a result of protein malnutrition. The pathogenic mechanism appears to be impairment of cellular immunity probably enhanced by concomitant impairment of humoral antibody immunity resulting also in decreased resistance to pyogenic and other secondary pathogens. The tissue edema attendant on decreased serum osmotic pressure due to lowering of the serum protein fractions enhances the probability of ulceration.
=============================================================
12.) Dermal ultrastructure in leprosy. =============================================================
ARTICLE SOURCE: Arch Pathol Lab Med (United States), May 1984, 108(5)
p383-6
AUTHOR(S): Van Hale HM; Turkel SB; Rea TH
PUBLICATION TYPE: JOURNAL ARTICLE
ABSTRACT: We studied the ultrastructure of the dermal inflammatory
response in 18 patients with leprosy. Biopsy specimens from 14 lepromatous patients, including four with Lucio's phenomenon and four with erythema nodosum leprosum, were compared with biopsy specimens from one borderline lepromatous and three borderline tuberculoid patients. In all, the dermal infiltrate consisted of macrophages, lymphocytes, and mast cells.
This infiltrate was predominantly perivascular, and chronic reactive changes were found in the small dermal vessels. The macrophages contained phagocytized organisms within membrane-bound vacuoles and a wide variety of lysosomal residual dense bodies. Intraendothelial organisms were occasionally seen, especially in biopsy specimens from the patients with Lucio's phenomenon. The greatest number of mast cells were also seen in the infiltrate in those cases. The frequent close association of macrophages with lymphocytes and mast cells suggests an interrelationship between these cells that appears typical of the host response to leprosy.
=============================================================
13.) Lepromatous and tuberculoid leprosy: clinical presentation and cytokine responses.
=============================================================
Ochoa MT; Valderrama L; Ochoa A; Zea A; Escobar CE; Moreno LH; Falabella Dermatology Service, Universidad del Valle, Cali, Colombia.
Int J Dermatol (UNITED STATES) Nov 1996 35 (11) p786-90
OBJECTIVE:
This study analyzes the major clinical characteristics of patients with active leprosy in relation to the in vitro immune response to the T-lymphocyte activator anti-CD3.
METHODS:
Thirty-eight patients with an established diagnosis of leprosy were classified according to the Ridley and Jopling table. Peripheral blood mononuclear cells from both lepromatous leprosy (LL) and tuberculoid leprosy (TL) patients and healthy controls were used to evaluate lymphocyte proliferation; immunoenzymatic assays were used to evaluate cytokine production (IL-1, IL-2, IL-4, IL-6, IL-10, IFN-gamma).
RESULTS:
Peripheral blood mononuclear cells from both LL and TL patients displayed blastogenic responses to anti-CD3. The cytokines IL-1 beta, IL-6, IL-10, and IFN-gamma were detected in culture supernatants. Endogenous production of IL-1 beta was significantly higher in cell cultures from patients with the lepromatous form of the disease compared to those with tuberculoid leprosy. Production of IL-6 in response to anti-CD3 was observed in a significantly higher proportion of LL than TL patients (P = 0.0025). Gamma-interferon production did not differ between TL and LL, but a direct correlation was observed between time of multidrug treatment and IFN production in vitro (P = 0.016). Interleukin-10 was detected in culture supernatants of lymphocytes activated by anti-CD3 from both patient groups, but not from healthy controls.
CONCLUSIONS:
The findings of this study suggest that patients with the two distinct forms of leprosy are capable of responding to a polyclonal T-lymphocyte stimulus such as anti-CD3 and provide evidence suggestive of alterations in the immune responses mediated by cytokines that may contribute to the spectrum of disease and response to treatment.
=============================================================
14.) Leprosy (Hansen's disease) in South Dakota.
=============================================================
S D J Med 1996 Jun;49(6):185-7 Related Articles, Books, LinkOut
Burrish G, Hartmann A, Lockwood W
Department of Dermatology, Central Plains Clinic, Sioux Falls, SD, USA.
Worldwide Hansen's disease is an important and relatively common disease, but is still very rare in South Dakota. Two patients are described to help demonstrate the wide variety of clinical manifestations associated with Hansen's disease. Since the clinical appearance of Hansen's disease is highly variable, the following six forms of Hansen's disease are described: Indeterminate, tuberculoid (TT), borderline tuberculoid (BT), borderline (BB), borderline lepromatous (BL), and lepromatous leprosy (LL). In addition, three well-recognized reactional forms of leprosy are also described: Type 1 (lepra reaction), type 2 (erythema nodosum leprosum), and type 3 (Lucio's phenomenon). While the disease affects primarily the skin and nerves, health care providers of all disciplines should remain alert for this disease which can present with a high degree of clinical variability.
=============================================================
15.) [Virchowian Hansen's disease, Lucio's phenomenon, cryptococcosis].
=============================================================
Hansenol Int 1988 Dec;13(2):47-56 Related Articles, Books, LinkOut
[Article in Portugese]
A 75 years old white male, for 3 years on treatment for virchowian hanseniasis, was admitted with active HD lesions, infiltration on the base of right lung, leg ulcer and malaise. After two days he developed purpura and hemorrhagic blisters in the limbs. The biopsy of these lesions revealed Lucio phenomenon. The patient worsened with mental confusion, psychomotor agitation and anisocoric pupils. In the 18th day of internation the patient died. Necropsy revealed virchowian infiltration plenty of bacilli in the skin and viscera as well as tuberculoid granuloma with acid-fast bacilli in the liver, spleen and bone marrow. These findings lead us to review the patient's classification from virchowian to borderline. In the lungs, leptomeninge, renal papile, prostate and thyroid it was found loose tuberculoid granuloma with a great amount of fungi surrounded by a gelly halo resembling Criptococcus neoformans. These findings and the onset of Lucio phenomenon are discussed in a patient that has been treated for 3 years and still having several virchowian lesions and a great amount of acid-fast bacilli.
=============================================================
16.) Erythema nodosum leprosum in Singapore.
=============================================================
Ann Acad Med Singapore 1987 Oct;16(4):658-62
Giam YC, Ong BH, Tan T
Middle Road Hospital, Singapore.
Erythema Nodosum Leprosum (ENL) or Type II reaction is an immune complex syndrome seen in multibacillary leprosy. 20 patients with histological confirmation of ENL in leprosy were studied from 1982 to 1986. These patients had a range of clinical signs, from fever, tender dusky nodules, bullae, ulcers to lymphadenopathy, arthralgia and neuritis. The four major histological patterns are: a) classical pattern showing heavy infiltrations of neutrophils in three cases, b) sub-epidermal bulla pattern with marked oedema of the upper dermis, and collections of neutrophils in five cases, c) vasculitis pattern, affecting superficial and mid-dermal vessels, leading to epidermal necrosis, bulla formation and ulceration. Dilated vessels, congestion, lumenal fibrin clots and fibrinoid necrosis of vessels were seen, d) non-specific picture in nine cases with mild oedema, infiltration with neutrophils, and two cases with minimal reaction had chronic ENL with clinical vasculitis. All the five cases with vasculitis showed C1q, C3 and fibrinogen in the vessels. Comparing ENL reactions reported in Asia, our pattern is similar to that of Malaysians with the majority showing sub-epidermal oedema. Vasculitis is more common in India. Oedema with collagen necrosis as seen in acute ENL with iritis in New Guinea. The Lucio's phenomenon was not seen in any of the countries in Asia.
=============================================================
17.) Epidermal keratinocyte Ia expression, Langerhans cell hyperplasia and lymphocytic infiltration in skin lesions of leprosy.
=============================================================
Clin Exp Immunol 1986 Aug;65(2):253-9 Related Articles, Books, LinkOut
Rea TH, Shen JY, Modlin RL
Epidermal changes, Ia expression on keratinocytes, Langerhans cell hyperplasia and lymphocyte infiltration were sought in skin lesions of leprosy: 15 borderline tuberculoid (BT), six borderline lepromatous (BL), 17 lepromatous (LL), 13 erythema nodosum leprosum (ENL), six Lucio reactions and nine reversal reactions. All three changes were well developed in BT and reversal reactions. ENL showed well developed keratinocyte Ia and Langerhans cell hyperplasia, but little lymphocytic infiltration. LL and Lucio tissues had some Langerhans cell hyperplasia but little or no keratinocyte Ia or lymphocytic infiltration. BL tissues were so diverse as to suggest two distinct subgroups. These findings are consistent with the hypothesis that keratinocyte Ia expression is an immunohistological sign of a cell-mediated immune (CMI) response. However, the Ia keratinocyte expression found in BL and ENL tissues appears contrary to the undifferentiated macrophages and numerous bacilli found in the lesions. Thus, if a sign of CMI, keratinocyte Ia expression is not a measure of the effectiveness of the response.
=============================================================
18.) Specific antigen and antibody to Mycobacterium leprae in the cryoprecipitate of a patient with Lucio phenomenon.
=============================================================
Rheumatol Int 1986;6(2):93-4 Related Articles, Books, LinkOut
Drosos AA, Brennan PJ, Elisaf MS, Stefanou SG, Papadimitriou CS,
Moutsopoulos HM
Using a sensitive and specific enzyme-linked immunosorbent assay (ELISA) assay we showed that the cryoglobulins of a patient with Lucio phenomenon contain phenolic glycolipid I antigen and a specific antibody.
=============================================================
19.) In situ characterization of T lymphocyte subsets in the reactional
states of leprosy.
=============================================================
Clin Exp Immunol 1983 Jul;53(1):17-24 Related Articles, Books, LinkOut
Modlin RL, Gebhard JF, Taylor CR, Rea TH
Using monoclonal antibodies and the immunoperoxidase technique, the numbers and distribution of T lymphocyte subsets in the tissues of reactional states of leprosy (six reversal reaction, nine erythema nodosum leprosum (ENL) and two Lucio's reaction) were determined and compared with those found in stable, non-reactional patients (six tuberculoid, two borderline lepromatous and seven lepromatous).
The pattern of segregation of the suppressor/cytotoxic phenotype at the periphery of the granuloma was found in both non-reactional tuberculoid lesions and reversal reactions, but was better developed in the former. In ENL and Lucio's reaction, as well as in non-reactional lepromatous tissue, the helper/inducer and suppressor/cytotoxic phenotypes were both admixed with the aggregated histiocytes. However, the helper/suppressor ratio in ENL (2.1 +/- 0.4) was significantly larger than that in non-reactional lepromatous tissue (0.7 +/- 0.4, P less than 0.001). The immature thymocyte antigen OKT6 was found on scattered large non-lymphoid cells, most commonly in tuberculoid and reversal reaction tissues, less commonly in ENL, but only irregularly in non-reactional lepromatous tissue.
The peripheral pattern of the suppressor/cytotoxic phenotype may be an immunohistological reflection of a cell-mediated immune response common to both non-reactional tuberculoid and reversal reaction patients. The reversal of the helper/suppressor ratio in ENL as compared to non-reactional lepromatous disease suggests some role for cell-mediated immunity in the pathogenesis of ENL. The OKT6 positive cell is of unknown origin and function.
=============================================================
20.) Ultrastructure of the dermal microvasculature in leprosy. =============================================================
Int J Lepr Other Mycobact Dis 1982 Jun;50(2):164-71
Turkel SB, Van Hale HM, Rea TH
Infection with M. leprae may lead to the presence of the organism within the dermal vascular endothelium, a phenomenon most pronounced in lepromatous leprosy. In order to study the ultrastructural features of the dermal microvasculature in leprosy, biopsies from 18 patients with lepromatous (14), borderline lepromatous (1) and borderline tuberculoid (3) leprosy were examined. Four patients with Lucio's phenomenon and four with erythema nodosum leprosum were included.
The ultrastructural changes in the dermal microvasculature included endothelial swelling and hypertrophy, increased endothelial and pericytic cytoplasmic processes, and pronounced basal lamina reduplication. Occasional large, pale, endothelial cells with widely dispersed organelles were encountered. Phagocytized, membrane-bound intraendothelial organisms were found, similar in appearance to those within dermal macrophages.
The predominantly perivascular dermal inflammatory infiltrate consisted of lymphocytes, macrophages and mast cells. The observed ultrastructural changes in the dermal microvasculature are similar to those previously described in the endoneurial vessels. While reflecting nonspecific responses of the dermal microvasculature in chronic inflammation, the findings support a possible role of the small dermal vessels in the chronic nature of the host's response to infection with M. leprae.
=============================================================
21.) Lucio's phenomenon: a comparative histological study. =============================================================
Int J Lepr Other Mycobact Dis 1979 Jun;47(2):161-6
Rea TH, Ridley DS
To study further the pathogenesis of Lucio's phenomenon, we have made a comparative histological study of 11 patients with Lucio's phenomenon and 12 with ENL. Confirming the findings of others, Lucio's reaction could be distinguished from ENL by epidermal necrosis and by necrotizing vasculitis manifesting necrosis in the walls of superficial vessels and severe, focal endothelial proliferation of mid-dermal vessels.
Furthermore, in Lucio's phenomenon large numbers of AFB were found in evidently normal and in swollen or proliferating endothelial cells. We hypothesize that patients with Lucio's phenomenon have an exceptionally deficient defense mechanism, allowing unrestricted proliferation of AFB in endothelial cells, facilitating contact between bacterial antigen and circulating antibody and leading to infarction; also, this nadir of resistance allows unimpeded dissemination of AFB, accounting for the clinical features of diffuse non-nodular leprosy. Thus, an explanation is offered for the restriction of Lucio's phenomenon to patients with diffuse non-nodular lepromatous leprosy.
=============================================================
22.) Serum macrophage migration inhibition activity in patients with leprosy.
=============================================================
J Invest Dermatol 1982 Nov;79(5):336-9
Rea TH, Yoshida T
We have found that 26 of 54 (48%) untreated patients with leprosy had serum migration inhibitory activity, and that this was present in tuberculoid, borderline, and lepromatous forms of the disease. Patients with active recreational states; i.e., reversal reactions, Lucio's reaction, or erythema nodosum leprosum, were particularly apt to have this inhibitory activity. The prevalence of inhibitory activity did not vary significantly with treatment, dinitrochlorobenzene responsiveness, tuberculin responsiveness, or serum lysozyme levels.
=============================================================
23.) [Leprosy tests: diagnostic problems].
Ann Acad Med Singapore 1987 Oct;16(4):658-62
Giam YC, Ong BH, Tan T
Middle Road Hospital, Singapore.
Erythema Nodosum Leprosum (ENL) or Type II reaction is an immune complex syndrome seen in multibacillary leprosy. 20 patients with histological confirmation of ENL in leprosy were studied from 1982 to 1986. These patients had a range of clinical signs, from fever, tender dusky nodules, bullae, ulcers to lymphadenopathy, arthralgia and neuritis. The four major histological patterns are: a) classical pattern showing heavy infiltrations of neutrophils in three cases, b) sub-epidermal bulla pattern with marked oedema of the upper dermis, and collections of neutrophils in five cases, c) vasculitis pattern, affecting superficial and mid-dermal vessels, leading to epidermal necrosis, bulla formation and ulceration. Dilated vessels, congestion, lumenal fibrin clots and fibrinoid necrosis of vessels were seen, d) non-specific picture in nine cases with mild oedema, infiltration with neutrophils, and two cases with minimal reaction had chronic ENL with clinical vasculitis. All the five cases with vasculitis showed C1q, C3 and fibrinogen in the vessels. Comparing ENL reactions reported in Asia, our pattern is similar to that of Malaysians with the majority showing sub-epidermal oedema. Vasculitis is more common in India. Oedema with collagen necrosis as seen in acute ENL with iritis in New Guinea. The Lucio's phenomenon was not seen in any of the countries in Asia.
=============================================================
17.) Epidermal keratinocyte Ia expression, Langerhans cell hyperplasia and lymphocytic infiltration in skin lesions of leprosy.
=============================================================
Clin Exp Immunol 1986 Aug;65(2):253-9 Related Articles, Books, LinkOut
Rea TH, Shen JY, Modlin RL
Epidermal changes, Ia expression on keratinocytes, Langerhans cell hyperplasia and lymphocyte infiltration were sought in skin lesions of leprosy: 15 borderline tuberculoid (BT), six borderline lepromatous (BL), 17 lepromatous (LL), 13 erythema nodosum leprosum (ENL), six Lucio reactions and nine reversal reactions. All three changes were well developed in BT and reversal reactions. ENL showed well developed keratinocyte Ia and Langerhans cell hyperplasia, but little lymphocytic infiltration. LL and Lucio tissues had some Langerhans cell hyperplasia but little or no keratinocyte Ia or lymphocytic infiltration. BL tissues were so diverse as to suggest two distinct subgroups. These findings are consistent with the hypothesis that keratinocyte Ia expression is an immunohistological sign of a cell-mediated immune (CMI) response. However, the Ia keratinocyte expression found in BL and ENL tissues appears contrary to the undifferentiated macrophages and numerous bacilli found in the lesions. Thus, if a sign of CMI, keratinocyte Ia expression is not a measure of the effectiveness of the response.
=============================================================
18.) Specific antigen and antibody to Mycobacterium leprae in the cryoprecipitate of a patient with Lucio phenomenon.
=============================================================
Rheumatol Int 1986;6(2):93-4 Related Articles, Books, LinkOut
Drosos AA, Brennan PJ, Elisaf MS, Stefanou SG, Papadimitriou CS,
Moutsopoulos HM
Using a sensitive and specific enzyme-linked immunosorbent assay (ELISA) assay we showed that the cryoglobulins of a patient with Lucio phenomenon contain phenolic glycolipid I antigen and a specific antibody.
=============================================================
19.) In situ characterization of T lymphocyte subsets in the reactional
states of leprosy.
=============================================================
Clin Exp Immunol 1983 Jul;53(1):17-24 Related Articles, Books, LinkOut
Modlin RL, Gebhard JF, Taylor CR, Rea TH
Using monoclonal antibodies and the immunoperoxidase technique, the numbers and distribution of T lymphocyte subsets in the tissues of reactional states of leprosy (six reversal reaction, nine erythema nodosum leprosum (ENL) and two Lucio's reaction) were determined and compared with those found in stable, non-reactional patients (six tuberculoid, two borderline lepromatous and seven lepromatous).
The pattern of segregation of the suppressor/cytotoxic phenotype at the periphery of the granuloma was found in both non-reactional tuberculoid lesions and reversal reactions, but was better developed in the former. In ENL and Lucio's reaction, as well as in non-reactional lepromatous tissue, the helper/inducer and suppressor/cytotoxic phenotypes were both admixed with the aggregated histiocytes. However, the helper/suppressor ratio in ENL (2.1 +/- 0.4) was significantly larger than that in non-reactional lepromatous tissue (0.7 +/- 0.4, P less than 0.001). The immature thymocyte antigen OKT6 was found on scattered large non-lymphoid cells, most commonly in tuberculoid and reversal reaction tissues, less commonly in ENL, but only irregularly in non-reactional lepromatous tissue.
The peripheral pattern of the suppressor/cytotoxic phenotype may be an immunohistological reflection of a cell-mediated immune response common to both non-reactional tuberculoid and reversal reaction patients. The reversal of the helper/suppressor ratio in ENL as compared to non-reactional lepromatous disease suggests some role for cell-mediated immunity in the pathogenesis of ENL. The OKT6 positive cell is of unknown origin and function.
=============================================================
20.) Ultrastructure of the dermal microvasculature in leprosy. =============================================================
Int J Lepr Other Mycobact Dis 1982 Jun;50(2):164-71
Turkel SB, Van Hale HM, Rea TH
Infection with M. leprae may lead to the presence of the organism within the dermal vascular endothelium, a phenomenon most pronounced in lepromatous leprosy. In order to study the ultrastructural features of the dermal microvasculature in leprosy, biopsies from 18 patients with lepromatous (14), borderline lepromatous (1) and borderline tuberculoid (3) leprosy were examined. Four patients with Lucio's phenomenon and four with erythema nodosum leprosum were included.
The ultrastructural changes in the dermal microvasculature included endothelial swelling and hypertrophy, increased endothelial and pericytic cytoplasmic processes, and pronounced basal lamina reduplication. Occasional large, pale, endothelial cells with widely dispersed organelles were encountered. Phagocytized, membrane-bound intraendothelial organisms were found, similar in appearance to those within dermal macrophages.
The predominantly perivascular dermal inflammatory infiltrate consisted of lymphocytes, macrophages and mast cells. The observed ultrastructural changes in the dermal microvasculature are similar to those previously described in the endoneurial vessels. While reflecting nonspecific responses of the dermal microvasculature in chronic inflammation, the findings support a possible role of the small dermal vessels in the chronic nature of the host's response to infection with M. leprae.
=============================================================
21.) Lucio's phenomenon: a comparative histological study. =============================================================
Int J Lepr Other Mycobact Dis 1979 Jun;47(2):161-6
Rea TH, Ridley DS
To study further the pathogenesis of Lucio's phenomenon, we have made a comparative histological study of 11 patients with Lucio's phenomenon and 12 with ENL. Confirming the findings of others, Lucio's reaction could be distinguished from ENL by epidermal necrosis and by necrotizing vasculitis manifesting necrosis in the walls of superficial vessels and severe, focal endothelial proliferation of mid-dermal vessels.
Furthermore, in Lucio's phenomenon large numbers of AFB were found in evidently normal and in swollen or proliferating endothelial cells. We hypothesize that patients with Lucio's phenomenon have an exceptionally deficient defense mechanism, allowing unrestricted proliferation of AFB in endothelial cells, facilitating contact between bacterial antigen and circulating antibody and leading to infarction; also, this nadir of resistance allows unimpeded dissemination of AFB, accounting for the clinical features of diffuse non-nodular leprosy. Thus, an explanation is offered for the restriction of Lucio's phenomenon to patients with diffuse non-nodular lepromatous leprosy.
=============================================================
22.) Serum macrophage migration inhibition activity in patients with leprosy.
=============================================================
J Invest Dermatol 1982 Nov;79(5):336-9
Rea TH, Yoshida T
We have found that 26 of 54 (48%) untreated patients with leprosy had serum migration inhibitory activity, and that this was present in tuberculoid, borderline, and lepromatous forms of the disease. Patients with active recreational states; i.e., reversal reactions, Lucio's reaction, or erythema nodosum leprosum, were particularly apt to have this inhibitory activity. The prevalence of inhibitory activity did not vary significantly with treatment, dinitrochlorobenzene responsiveness, tuberculin responsiveness, or serum lysozyme levels.
=============================================================
23.) [Leprosy tests: diagnostic problems].
=============================================================
Acta Leprol 1981 Apr-Jun;(83):11-9 Related Articles, Books, LinkOut
[Article in French]
Strobel M, Ndiaye B, Marchand JP, Stach JL, Foumoux F
Two cases of reactional leprosy leading to wrong diagnosis are reported. The first one concerns a reversal reaction predominantly neuritic, initially taken for polyarthritis. The second one concerns an erythema nodosum leprosum with extensive cutaneous necrosis (Lucio's phenomenon or ulcerative lazarine leprosy). Main aspects and mechanisms of leprosy reactional states are reviewed. It is emphasized that errors or delays in diagnosis are often caused by failing to recognize cutaneous or neuritic symptoms.
=============================================================
24.) Serum and tissue lysozyme in leprosy.
=============================================================
Infect Immun 1977 Dec;18(3):847-56 Related Articles, Books, LinkOut
Rea TH, Taylor CR
Mean serum lysozyme values were found to be elevated in untreated leprosy patients. Statistically significant elevations were present in each of the three major categories of leprosy, tuberculoid, borderline, and lepromatous. Values were particularly high in patients with severe reversal reactions or Lucio's phenomenon. Prolonged sulfone therapy was associated with a fall in serum lysozyme values. With an immunoperoxidase method to localize lysozyme in leprous tissues, two distinct staining patterns were found, granular and saccular.
The grandular pattern of lysozymal staining was found in epithelioid cells and in giant cells, and the intensity of staining showed a positive correlation with serum lysozyme levels. Conversely, a saccular pattern of lysozymal staining was found in lepromatous histiocytes, buth the intensity of staining was unrelated to serum lysozyme levels; the saccular structures contained dense aggregates of Mycobacterium leprae.
These two patterns of staining probably represent different functional responses of monocyte-derived granuloma cells, whereas the serum levels reflect, to a varying degree, both the absolute number of such cells and the rate of secretory activity of this cell population as a whole.
=============================================================
25.) [Reactional status of leprosy].
Acta Leprol 1981 Apr-Jun;(83):11-9 Related Articles, Books, LinkOut
[Article in French]
Strobel M, Ndiaye B, Marchand JP, Stach JL, Foumoux F
Two cases of reactional leprosy leading to wrong diagnosis are reported. The first one concerns a reversal reaction predominantly neuritic, initially taken for polyarthritis. The second one concerns an erythema nodosum leprosum with extensive cutaneous necrosis (Lucio's phenomenon or ulcerative lazarine leprosy). Main aspects and mechanisms of leprosy reactional states are reviewed. It is emphasized that errors or delays in diagnosis are often caused by failing to recognize cutaneous or neuritic symptoms.
=============================================================
24.) Serum and tissue lysozyme in leprosy.
=============================================================
Infect Immun 1977 Dec;18(3):847-56 Related Articles, Books, LinkOut
Rea TH, Taylor CR
Mean serum lysozyme values were found to be elevated in untreated leprosy patients. Statistically significant elevations were present in each of the three major categories of leprosy, tuberculoid, borderline, and lepromatous. Values were particularly high in patients with severe reversal reactions or Lucio's phenomenon. Prolonged sulfone therapy was associated with a fall in serum lysozyme values. With an immunoperoxidase method to localize lysozyme in leprous tissues, two distinct staining patterns were found, granular and saccular.
The grandular pattern of lysozymal staining was found in epithelioid cells and in giant cells, and the intensity of staining showed a positive correlation with serum lysozyme levels. Conversely, a saccular pattern of lysozymal staining was found in lepromatous histiocytes, buth the intensity of staining was unrelated to serum lysozyme levels; the saccular structures contained dense aggregates of Mycobacterium leprae.
These two patterns of staining probably represent different functional responses of monocyte-derived granuloma cells, whereas the serum levels reflect, to a varying degree, both the absolute number of such cells and the rate of secretory activity of this cell population as a whole.
=============================================================
25.) [Reactional status of leprosy].
=============================================================
Med Cutan Ibero Lat Am 1975;3(3):199-208 Related Articles, Books
Alonso AM
Reactional leprosy is studied according to its clinical forms
A) Lepromatous a) Acute lepromatization: encroaching and invasive nature; the patient becomes more and more lepromatous ; bad prognosis. b) Erythema nodosum: "contusiform dermatitis"; variable prognosis not so bad as it is in the preceding case; allergic nature and its evolution is usually detained and therapeutics efficient. c) Erythema multiform. d) Lucio's phenomenon: vascular lesions and consequently necrosis as a complication of the "erythema necrotisans" (beautiful leprosy).
B) Tuberculoid Reactional tuberculoid is the only one in this benign type, the Mitsuda's test must always be positive and prognosis consequently good.
C) Dimorphous or "Borderline" whose Mitsuda's test is mostly negative, sometimes positive, but not stable. The lesions may stimulate the tuberculoid leprids but they invade mucous membranes, are impregnated by pigmentation, may present the Unna's band, and other characteristics of the Lepromatous type. Are associated (fever, asthenia and emaciation). Prognosis not very good, because of the possibility of lepromatization, according to its tendency. Evolution slower and frequent relapses. Besides there are nodular lesions. Pathogeny
1) Perifocal allergic reaction (Jadassohn). Similar to epituberculosis and Herxheimer reaction.
2) Septicemia. Sensitized tissues inside or outside the lesions, are invaded by the bacilli and so the allergic reaction takes place. Even without culture resources, Mycobacterium leprae has been found in the blood by direct examination.
3) Autoimmunization (Waldenstrom, Matthews and Trantman, 1965).
Based upon the similarity between both humoral syndromes, in leprosy reactions and collagenous, diseases, as to: hypergammaglobulins, hypercryoproteins, antigammaglobulins, serological reactions (Wassermann, Kahn, Kline, VDRL) positives, Antistreptolysin O, protein C reactive, antinuclear factors, latex and Wadler-Rose test positives (rheumatoid tests) lowering of complement.
If leprosy reaction is like this, it should be the less agressive of the autoimmune diseases. a) Its eruptions are cyclic not of long standing duration, as a general rule. b) Its prognosis has been recognized as good, except lately, because of the use of corticoid therapy which has been fatal, in many cases. After some years the leprosy reaction cures spontaneously. Treatment (see article)
=============================================================
26.) Auricular chondritis as a rheumatologic manifestation of Lucio's phenomenon: clinical improvement after plasmapheresis. =============================================================
Ann Intern Med 1983 Jan;98(1):49-51
Piepkorn M, Brown C, Zone J
=============================================================
=============================================================
27.) Contemplative immune mechanism of Lucio phenomenon and its global status.
=============================================================
J Dermatol 1987 Dec;14(6):580-5
Sehgal VN, Srivastava G, Sharma VK
=============================================================
=============================================================
28.) Plasma exchange therapy in Lucio's phenomenon.
=============================================================
Arch Dermatol 1980 Oct;116(10):1101 Related Articles, Books, LinkOut
Wallach D, Cottenot F, Bussel A, Palangie A, Pennec J
Publication Types:
Letter
=============================================================
=============================================================
29.) [Lepromatous leprosy with extensive ulcerations and cachexia. The
Lucio phenomenon? Lazarine leprosy]?
=============================================================
Acta Leprol 1979 Sep-Dec;(76-77):331-3
[Article in French]
Strobel M, Ndiaye B, Carayon A
=============================================================
30. [2 cases of Lucio phenomenon in Paraguay].
=============================================================
Repura 1973 Jan-Mar;42(1):12-5
Innami S, Legiuzamon OR, Alvarenga AE
=============================================================
=============================================================
31.) An unusual case of leprosy with pathological features common to
Lucio's phenomenon.
=============================================================
Cent Afr J Med 1971 Jun;17(6):119-22
Taube E, Ellis BP
=============================================================
=============================================================
32.) Primary diffuse lepromatous leprosy with erythema necrotisans
(lucio phenomenon).
=============================================================
Arch Dermatol 1968 May;97(5):593-4
Moschella SL
=============================================================
=============================================================
33.) The "Lucio phenomenon" in diffuse leprosy.
=============================================================
Ann Intern Med 1967 Oct;67(4):831-6
Donner RS, Shively JA
=============================================================
=============================================================
34.) [Macular leprosy of Lucio--antimalarials in leprotic reaction].
=============================================================
Dermatol Int 1965 Jul-Sep;4(3):147-50
Padilla HC
=============================================================
=============================================================
35.) [Dermatology in the Central American tropics. I. Lucio's spotted
leprosy. Antimalarials in the leprous reaction].
=============================================================
Rev Med Hondur 1965 Jul-Sep;33(3):129-35
Corrales Padilla H
=============================================================
=============================================================
36.) [Lucio's leprosy].
=============================================================
Division de Estudios de Posgrado e Investigacion, Facultad de Medicina,
UNAM, Mexico, D.F.
Gac Med Mex 1996 May-Jun;132(3):333-4
Quijano-Pitman F
Publication Types:
Biography
Historical article
=============================================================
=============================================================
37.) [Lucio phenomenon in leprosy reactions].
=============================================================
Nippon Rai Gakkai Zasshi 1980 Apr-Jun;49(2):113-6
Mayama A
=============================================================
=============================================================
38.) Lucio's phenomenon: an overview.
=============================================================
Lepr Rev 1979 Jun;50(2):107-12
Rea TH
=============================================================
=============================================================
39.) Lucio's phenomenon: an immune complex deposition syndrome in
lepromatous leprosy.
=============================================================
Clin Immunol Immunopathol 1978 Feb;9(2):184-93
Quismorio FP Jr, Rea T, Chandor S, Levan N, Friou GJ
=============================================================
=============================================================
40.) [Lucio's leprosy].
=============================================================
Actas Dermosifiliogr 1976 Jan-Feb;67(1-2):31-6
Nunez Moreno A, Sotillo Gago I, Castro Romero A, Lopez Molina M
=============================================================
===========================================================
41.) Antiphospholipid antibodies thrombotic syndrome misdiagnosed as Lucio's phenomenon.
=============================================================
Int J Lepr Other Mycobact Dis 1996 Sep;64(3):320-3
Bakos L, Correa CC, Bergmann L, Bonamigo RR, Muller LF
Department of Internal Medicine, Hospital de Clinicas de Porto Alegre, Federal University of Rio Grande do Sul, Porto Alegre, Brazil. 30.)[2 cases of Lucio phenomenon in Paraguay].
=======================================================
Med Cutan Ibero Lat Am 1975;3(3):199-208 Related Articles, Books
Alonso AM
Reactional leprosy is studied according to its clinical forms
A) Lepromatous a) Acute lepromatization: encroaching and invasive nature; the patient becomes more and more lepromatous ; bad prognosis. b) Erythema nodosum: "contusiform dermatitis"; variable prognosis not so bad as it is in the preceding case; allergic nature and its evolution is usually detained and therapeutics efficient. c) Erythema multiform. d) Lucio's phenomenon: vascular lesions and consequently necrosis as a complication of the "erythema necrotisans" (beautiful leprosy).
B) Tuberculoid Reactional tuberculoid is the only one in this benign type, the Mitsuda's test must always be positive and prognosis consequently good.
C) Dimorphous or "Borderline" whose Mitsuda's test is mostly negative, sometimes positive, but not stable. The lesions may stimulate the tuberculoid leprids but they invade mucous membranes, are impregnated by pigmentation, may present the Unna's band, and other characteristics of the Lepromatous type. Are associated (fever, asthenia and emaciation). Prognosis not very good, because of the possibility of lepromatization, according to its tendency. Evolution slower and frequent relapses. Besides there are nodular lesions. Pathogeny
1) Perifocal allergic reaction (Jadassohn). Similar to epituberculosis and Herxheimer reaction.
2) Septicemia. Sensitized tissues inside or outside the lesions, are invaded by the bacilli and so the allergic reaction takes place. Even without culture resources, Mycobacterium leprae has been found in the blood by direct examination.
3) Autoimmunization (Waldenstrom, Matthews and Trantman, 1965).
Based upon the similarity between both humoral syndromes, in leprosy reactions and collagenous, diseases, as to: hypergammaglobulins, hypercryoproteins, antigammaglobulins, serological reactions (Wassermann, Kahn, Kline, VDRL) positives, Antistreptolysin O, protein C reactive, antinuclear factors, latex and Wadler-Rose test positives (rheumatoid tests) lowering of complement.
If leprosy reaction is like this, it should be the less agressive of the autoimmune diseases. a) Its eruptions are cyclic not of long standing duration, as a general rule. b) Its prognosis has been recognized as good, except lately, because of the use of corticoid therapy which has been fatal, in many cases. After some years the leprosy reaction cures spontaneously. Treatment (see article)
=============================================================
26.) Auricular chondritis as a rheumatologic manifestation of Lucio's phenomenon: clinical improvement after plasmapheresis. =============================================================
Ann Intern Med 1983 Jan;98(1):49-51
Piepkorn M, Brown C, Zone J
=============================================================
=============================================================
27.) Contemplative immune mechanism of Lucio phenomenon and its global status.
=============================================================
J Dermatol 1987 Dec;14(6):580-5
Sehgal VN, Srivastava G, Sharma VK
=============================================================
=============================================================
28.) Plasma exchange therapy in Lucio's phenomenon.
=============================================================
Arch Dermatol 1980 Oct;116(10):1101 Related Articles, Books, LinkOut
Wallach D, Cottenot F, Bussel A, Palangie A, Pennec J
Publication Types:
Letter
=============================================================
=============================================================
29.) [Lepromatous leprosy with extensive ulcerations and cachexia. The
Lucio phenomenon? Lazarine leprosy]?
=============================================================
Acta Leprol 1979 Sep-Dec;(76-77):331-3
[Article in French]
Strobel M, Ndiaye B, Carayon A
=============================================================
30. [2 cases of Lucio phenomenon in Paraguay].
=============================================================
Repura 1973 Jan-Mar;42(1):12-5
Innami S, Legiuzamon OR, Alvarenga AE
=============================================================
=============================================================
31.) An unusual case of leprosy with pathological features common to
Lucio's phenomenon.
=============================================================
Cent Afr J Med 1971 Jun;17(6):119-22
Taube E, Ellis BP
=============================================================
=============================================================
32.) Primary diffuse lepromatous leprosy with erythema necrotisans
(lucio phenomenon).
=============================================================
Arch Dermatol 1968 May;97(5):593-4
Moschella SL
=============================================================
=============================================================
33.) The "Lucio phenomenon" in diffuse leprosy.
=============================================================
Ann Intern Med 1967 Oct;67(4):831-6
Donner RS, Shively JA
=============================================================
=============================================================
34.) [Macular leprosy of Lucio--antimalarials in leprotic reaction].
=============================================================
Dermatol Int 1965 Jul-Sep;4(3):147-50
Padilla HC
=============================================================
=============================================================
35.) [Dermatology in the Central American tropics. I. Lucio's spotted
leprosy. Antimalarials in the leprous reaction].
=============================================================
Rev Med Hondur 1965 Jul-Sep;33(3):129-35
Corrales Padilla H
=============================================================
=============================================================
36.) [Lucio's leprosy].
=============================================================
Division de Estudios de Posgrado e Investigacion, Facultad de Medicina,
UNAM, Mexico, D.F.
Gac Med Mex 1996 May-Jun;132(3):333-4
Quijano-Pitman F
Publication Types:
Biography
Historical article
=============================================================
=============================================================
37.) [Lucio phenomenon in leprosy reactions].
=============================================================
Nippon Rai Gakkai Zasshi 1980 Apr-Jun;49(2):113-6
Mayama A
=============================================================
=============================================================
38.) Lucio's phenomenon: an overview.
=============================================================
Lepr Rev 1979 Jun;50(2):107-12
Rea TH
=============================================================
=============================================================
39.) Lucio's phenomenon: an immune complex deposition syndrome in
lepromatous leprosy.
=============================================================
Clin Immunol Immunopathol 1978 Feb;9(2):184-93
Quismorio FP Jr, Rea T, Chandor S, Levan N, Friou GJ
=============================================================
=============================================================
40.) [Lucio's leprosy].
=============================================================
Actas Dermosifiliogr 1976 Jan-Feb;67(1-2):31-6
Nunez Moreno A, Sotillo Gago I, Castro Romero A, Lopez Molina M
=============================================================
===========================================================
41.) Antiphospholipid antibodies thrombotic syndrome misdiagnosed as Lucio's phenomenon.
=============================================================
Int J Lepr Other Mycobact Dis 1996 Sep;64(3):320-3
Bakos L, Correa CC, Bergmann L, Bonamigo RR, Muller LF
Department of Internal Medicine, Hospital de Clinicas de Porto Alegre, Federal University of Rio Grande do Sul, Porto Alegre, Brazil. 30.)[2 cases of Lucio phenomenon in Paraguay].
=======================================================
42.) "Lucio's Phenomenon" Associated with Mycobacterium lepromatosis.=======================================================
Velarde-Félix JS1, Alvarado-Villa G2, Vera-Cabrera L2.
Author information
1
Centro de Medicina Genómica, Servicios de Salud de Sinaloa, Sinaloa, México; Unidad Académica Escuela de Biología, Universidad Autónoma de Sinaloa, Sinaloa, México; Centro de Salud Urbano de Guasave, Servicios de Salud de Sinaloa, Guasave, Sinaloa, Mexico; Servicio de Dermatología, Hospital Universitario, Universidad Autónoma de Nuevo León, Monterrey, Mexico jsvelfe@hotmail.com.
2
Centro de Medicina Genómica, Servicios de Salud de Sinaloa, Sinaloa, México; Unidad Académica Escuela de Biología, Universidad Autónoma de Sinaloa, Sinaloa, México; Centro de Salud Urbano de Guasave, Servicios de Salud de Sinaloa, Guasave, Sinaloa, Mexico; Servicio de Dermatología, Hospital Universitario, Universidad Autónoma de Nuevo León, Monterrey, Mexico.
A 49-year-old man born on Sinaloa, Mexico, presented to the consult on December 2014 with ulcerations in the abdominal region and lower extremities with serous fluid, foul smell, and pus, but without lymphadenopathy. Three years before he developed hypopigmented macules in scapular zone, and in November 2014, the first nodules in lower extremities appeared accompanied by fever and pain, as well as erythema nodosum leprosum; the latter was treated with thalidomide for 10 days.
Bacilloscopy of right earlobe (not shown) and Fite–Faraco stained skin (Figure 1A ) were positive to acid-fast bacilli. Furthermore, chronic granulomatous dermatitis and necrotizing cutaneous vasculitis, characteristic of Lucio's phenomenon (LP) (Figure 1B), were observed.
Author information
1
Centro de Medicina Genómica, Servicios de Salud de Sinaloa, Sinaloa, México; Unidad Académica Escuela de Biología, Universidad Autónoma de Sinaloa, Sinaloa, México; Centro de Salud Urbano de Guasave, Servicios de Salud de Sinaloa, Guasave, Sinaloa, Mexico; Servicio de Dermatología, Hospital Universitario, Universidad Autónoma de Nuevo León, Monterrey, Mexico jsvelfe@hotmail.com.
2
Centro de Medicina Genómica, Servicios de Salud de Sinaloa, Sinaloa, México; Unidad Académica Escuela de Biología, Universidad Autónoma de Sinaloa, Sinaloa, México; Centro de Salud Urbano de Guasave, Servicios de Salud de Sinaloa, Guasave, Sinaloa, Mexico; Servicio de Dermatología, Hospital Universitario, Universidad Autónoma de Nuevo León, Monterrey, Mexico.
A 49-year-old man born on Sinaloa, Mexico, presented to the consult on December 2014 with ulcerations in the abdominal region and lower extremities with serous fluid, foul smell, and pus, but without lymphadenopathy. Three years before he developed hypopigmented macules in scapular zone, and in November 2014, the first nodules in lower extremities appeared accompanied by fever and pain, as well as erythema nodosum leprosum; the latter was treated with thalidomide for 10 days.
Bacilloscopy of right earlobe (not shown) and Fite–Faraco stained skin (Figure 1A ) were positive to acid-fast bacilli. Furthermore, chronic granulomatous dermatitis and necrotizing cutaneous vasculitis, characteristic of Lucio's phenomenon (LP) (Figure 1B), were observed.
=======================================
43.) Lucio's phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka.
========================================
43.) Lucio's phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka.
========================================
Herath S1, Navinan MR2, Liyanage I3, Rathnayaka N4, Yudhishdran J5,
Fernando J6, Sirimanne G7, Kulatunga A8.
Author information
1
National Hospital of Sri Lanka, Colombo, Sri Lanka. sandaherath@yahoo.com.
2
National Hospital of Sri Lanka, Colombo, Sri Lanka. rayno.navinan@gmail.com.
3
National Hospital of Sri Lanka, Colombo, Sri Lanka. isurujith@gmail.com.
4
National Hospital of Sri Lanka, Colombo, Sri Lanka. enthusiasum@yahoo.com.
5
National Hospital of Sri Lanka, Colombo, Sri Lanka. yudhishdran@yahoo.com.
6
Department of Pathology, National Hospital of Sri Lanka, Colombo, Sri Lanka. janakiefernando@gmail.com.
7
National Hospital of Sri Lanka, Colombo, Sri Lanka. gangamps@gmail.com.
8
National Hospital of Sri Lanka, Colombo, Sri Lanka. kulatunga.aruna@yahoo.com.
Abstract
BACKGROUND:
Lucio's phenomenon is a rare manifestation of untreated leprosy which is seen almost exclusively in regions surrounding the Gulf of Mexico. Its occurrence elsewhere though documented is considered uncommon. We present a case of Lucio's phenomenon in a previously undiagnosed leprosy patient who presented to us with its classical skin manifestations.
CASE PRESENTATION:
A 64 year old South Asian (Sri Lankan) male with a history of chronic obstructive airway disease presented to us with fever and cough. He had a generalized smooth and shiny skin with ulcerating skin lesions afflicting the digits of the fingers. The lesions progressed to involve the extremities of the body and healed with crusting. Based on the clinical and investigational findings Tuberculosis and common vasculitic conditions were suspected and excluded. The unusual skin manifestations prompted a biopsy, and wade fite stained revealed Mycobacterium bacilli. In context of the clinical picture and histological findings, Lucio's phenomenon was suspected. A clinical diagnosis of Lucio's phenomenon occurring in the backdrop of lepromatous leprosy was made.
CONCLUSION:
Though leprosy is still a prevalent disease, it has manifestations that are not easily recognized or fully appreciated. Regional patterns of atypical manifestations should not limit better understanding of rarer manifestations as it will aid in clinching an early diagnosis and instituting prompt treatment, thereby reducing morbidity and mortality.
Author information
1
National Hospital of Sri Lanka, Colombo, Sri Lanka. sandaherath@yahoo.com.
2
National Hospital of Sri Lanka, Colombo, Sri Lanka. rayno.navinan@gmail.com.
3
National Hospital of Sri Lanka, Colombo, Sri Lanka. isurujith@gmail.com.
4
National Hospital of Sri Lanka, Colombo, Sri Lanka. enthusiasum@yahoo.com.
5
National Hospital of Sri Lanka, Colombo, Sri Lanka. yudhishdran@yahoo.com.
6
Department of Pathology, National Hospital of Sri Lanka, Colombo, Sri Lanka. janakiefernando@gmail.com.
7
National Hospital of Sri Lanka, Colombo, Sri Lanka. gangamps@gmail.com.
8
National Hospital of Sri Lanka, Colombo, Sri Lanka. kulatunga.aruna@yahoo.com.
Abstract
BACKGROUND:
Lucio's phenomenon is a rare manifestation of untreated leprosy which is seen almost exclusively in regions surrounding the Gulf of Mexico. Its occurrence elsewhere though documented is considered uncommon. We present a case of Lucio's phenomenon in a previously undiagnosed leprosy patient who presented to us with its classical skin manifestations.
CASE PRESENTATION:
A 64 year old South Asian (Sri Lankan) male with a history of chronic obstructive airway disease presented to us with fever and cough. He had a generalized smooth and shiny skin with ulcerating skin lesions afflicting the digits of the fingers. The lesions progressed to involve the extremities of the body and healed with crusting. Based on the clinical and investigational findings Tuberculosis and common vasculitic conditions were suspected and excluded. The unusual skin manifestations prompted a biopsy, and wade fite stained revealed Mycobacterium bacilli. In context of the clinical picture and histological findings, Lucio's phenomenon was suspected. A clinical diagnosis of Lucio's phenomenon occurring in the backdrop of lepromatous leprosy was made.
CONCLUSION:
Though leprosy is still a prevalent disease, it has manifestations that are not easily recognized or fully appreciated. Regional patterns of atypical manifestations should not limit better understanding of rarer manifestations as it will aid in clinching an early diagnosis and instituting prompt treatment, thereby reducing morbidity and mortality.
=====================================
44.) Insight into the evolution and origin of leprosy bacilli from the genome sequence of Mycobacterium lepromatosis.
====================================
44.) Insight into the evolution and origin of leprosy bacilli from the genome sequence of Mycobacterium lepromatosis.
====================================
Proc Natl Acad Sci U S A. 2015 Apr 7;112(14):4459-64. doi:
10.1073/pnas.1421504112. Epub 2015 Mar 23.
Singh P1, Benjak A1, Schuenemann VJ2, Herbig A2, Avanzi C1, Busso P1, Nieselt K3, Krause J4, Vera-Cabrera L5, Cole ST6.
Author information
1
Global Health Institute, Ecole Polytechnique Fédérale de Lausanne, 1015 Lausanne, Switzerland;
2
Institute for Archaeological Sciences, University of Tübingen, 72076 Tübingen, Germany;
3
Center for Bioinformatics Tübingen, University of Tübingen, 72070 Tübingen, Germany;
4
Institute for Archaeological Sciences, University of Tübingen, 72076 Tübingen, Germany; Senckenberg Centre for Human Evolution and Palaeoenvironment, University of Tübingen, 72070 Tübingen, Germany; Max Planck Institute for the Science of Human History, 07745 Jena, Germany; and.
5
Laboratorio Interdisciplinario de Investigación Dermatológica, Servicio de Dermatología, Hospital Universitario, Universidad Autónoma de Nuevo León, 64460 Monterrey, Nuevo León, Mexico.
6
Global Health Institute, Ecole Polytechnique Fédérale de Lausanne, 1015 Lausanne, Switzerland; stewart.cole@epfl.ch.
Abstract
Mycobacterium lepromatosis is an uncultured human pathogen associated with diffuse lepromatous leprosy and a reactional state known as Lucio's phenomenon. By using deep sequencing with and without DNA enrichment, we obtained the near-complete genome sequence of M. lepromatosis present in a skin biopsy from a Mexican patient, and compared it with that of Mycobacterium leprae, which has undergone extensive reductive evolution. The genomes display extensive synteny and are similar in size (∼3.27 Mb). Protein-coding genes share 93% nucleotide sequence identity, whereas pseudogenes are only 82% identical. The events that led to pseudogenization of 50% of the genome likely occurred before divergence from their most recent common ancestor (MRCA), and both M. lepromatosis and M. leprae have since accumulated new pseudogenes or acquired specific deletions. Functional comparisons suggest that M. lepromatosis has lost several enzymes required for amino acid synthesis whereas M. leprae has a defective heme pathway. M. lepromatosis has retained all functions required to infect the Schwann cells of the peripheral nervous system and therefore may also be neuropathogenic. A phylogeographic survey of 227 leprosy biopsies by differential PCR revealed that 221 contained M. leprae whereas only six, all from Mexico, harbored M. lepromatosis. Phylogenetic comparisons indicate that M. lepromatosis is closer than M. leprae to the MRCA, and a Bayesian dating analysis suggests that they diverged from their MRCA approximately 13.9 Mya. Thus, despite their ancient separation, the two leprosy bacilli are remarkably conserved and still cause similar pathologic conditions.
Singh P1, Benjak A1, Schuenemann VJ2, Herbig A2, Avanzi C1, Busso P1, Nieselt K3, Krause J4, Vera-Cabrera L5, Cole ST6.
Author information
1
Global Health Institute, Ecole Polytechnique Fédérale de Lausanne, 1015 Lausanne, Switzerland;
2
Institute for Archaeological Sciences, University of Tübingen, 72076 Tübingen, Germany;
3
Center for Bioinformatics Tübingen, University of Tübingen, 72070 Tübingen, Germany;
4
Institute for Archaeological Sciences, University of Tübingen, 72076 Tübingen, Germany; Senckenberg Centre for Human Evolution and Palaeoenvironment, University of Tübingen, 72070 Tübingen, Germany; Max Planck Institute for the Science of Human History, 07745 Jena, Germany; and.
5
Laboratorio Interdisciplinario de Investigación Dermatológica, Servicio de Dermatología, Hospital Universitario, Universidad Autónoma de Nuevo León, 64460 Monterrey, Nuevo León, Mexico.
6
Global Health Institute, Ecole Polytechnique Fédérale de Lausanne, 1015 Lausanne, Switzerland; stewart.cole@epfl.ch.
Abstract
Mycobacterium lepromatosis is an uncultured human pathogen associated with diffuse lepromatous leprosy and a reactional state known as Lucio's phenomenon. By using deep sequencing with and without DNA enrichment, we obtained the near-complete genome sequence of M. lepromatosis present in a skin biopsy from a Mexican patient, and compared it with that of Mycobacterium leprae, which has undergone extensive reductive evolution. The genomes display extensive synteny and are similar in size (∼3.27 Mb). Protein-coding genes share 93% nucleotide sequence identity, whereas pseudogenes are only 82% identical. The events that led to pseudogenization of 50% of the genome likely occurred before divergence from their most recent common ancestor (MRCA), and both M. lepromatosis and M. leprae have since accumulated new pseudogenes or acquired specific deletions. Functional comparisons suggest that M. lepromatosis has lost several enzymes required for amino acid synthesis whereas M. leprae has a defective heme pathway. M. lepromatosis has retained all functions required to infect the Schwann cells of the peripheral nervous system and therefore may also be neuropathogenic. A phylogeographic survey of 227 leprosy biopsies by differential PCR revealed that 221 contained M. leprae whereas only six, all from Mexico, harbored M. lepromatosis. Phylogenetic comparisons indicate that M. lepromatosis is closer than M. leprae to the MRCA, and a Bayesian dating analysis suggests that they diverged from their MRCA approximately 13.9 Mya. Thus, despite their ancient separation, the two leprosy bacilli are remarkably conserved and still cause similar pathologic conditions.
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45.) Lucio Leprosy with Lucio's phenomenon, digital gangrene and anticardiolipin antibodies.
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45.) Lucio Leprosy with Lucio's phenomenon, digital gangrene and anticardiolipin antibodies.
==========================================
Nunzie E, Ortega Cabrera LV, Macanchi Moncayo FM, Ortega Espinosa PF,
Clapasson A, Massone C.
Abstract
Lucio's phenomenon (LPh) is considered a necrotizing panvasculitis and a variant of leprosy Type 2 reaction, clinically characterised by necrotic-haemorrhagic lesions on the extremities and trunk. LPh is observed in diffuse lepromatous leprosy (DLL or Lucio-Latapí leprosy). This is a distinct form of lepromatous leprosy (LL) reported mainly in Mexico. Anti-phospholipid antibody syndrome (APS) has been rarely described in LPh. We report a case of Lucio-Latapí leprosy with LPh observed in a patient from the province of El Oro in Ecuador, who presented clinical manifestations of long standing DLL (non-nodular infiltration of the skin, collapse of the nasal pyramid, madarosis, atrophy of the earlobes), of LPh (necrotic-haemorrhagic macules with irregular shapes) and of APS (necrosis of the right big and second toe). Histopathology showed perineural and periadnexal foamy macrophages with numerous bacilli (diagnostic of LL) in the subcutis, a mild lobular panniculitis with a large subcutaneous vessel infiltrated by macrophages in the wall (typical of LPh) and vessels of the superficial and mid dermis occluded by thrombi but without signs of vasculitis (typical of occlusive vasculopathy as in APS). Our observations suggest that some cases of LPh may be associated with APS. Anti-cardiolipin antibodies (aCL) and lupus anticoagulant (LA) should be tested in patients with LPh because this may have therapeutic implications.
Abstract
Lucio's phenomenon (LPh) is considered a necrotizing panvasculitis and a variant of leprosy Type 2 reaction, clinically characterised by necrotic-haemorrhagic lesions on the extremities and trunk. LPh is observed in diffuse lepromatous leprosy (DLL or Lucio-Latapí leprosy). This is a distinct form of lepromatous leprosy (LL) reported mainly in Mexico. Anti-phospholipid antibody syndrome (APS) has been rarely described in LPh. We report a case of Lucio-Latapí leprosy with LPh observed in a patient from the province of El Oro in Ecuador, who presented clinical manifestations of long standing DLL (non-nodular infiltration of the skin, collapse of the nasal pyramid, madarosis, atrophy of the earlobes), of LPh (necrotic-haemorrhagic macules with irregular shapes) and of APS (necrosis of the right big and second toe). Histopathology showed perineural and periadnexal foamy macrophages with numerous bacilli (diagnostic of LL) in the subcutis, a mild lobular panniculitis with a large subcutaneous vessel infiltrated by macrophages in the wall (typical of LPh) and vessels of the superficial and mid dermis occluded by thrombi but without signs of vasculitis (typical of occlusive vasculopathy as in APS). Our observations suggest that some cases of LPh may be associated with APS. Anti-cardiolipin antibodies (aCL) and lupus anticoagulant (LA) should be tested in patients with LPh because this may have therapeutic implications.
===============================
46.) Lucio's leprosy: a clinical and therapeutic challenge.
===============================
46.) Lucio's leprosy: a clinical and therapeutic challenge.
===============================
Jurado F1, Rodriguez O2, Novales J3, Navarrete G3, Rodriguez M2.
Author information
1
Department of Dermatology, Centro Dermatologico "Dr. Ladislao de la Pascua," Ministry of Health of the Government of The Federal District of Mexico City, Mexico. Electronic address: fermin_santac@yahoo.com.mx.
2
Department of Dermatology, Centro Dermatologico "Dr. Ladislao de la Pascua," Ministry of Health of the Government of The Federal District of Mexico City, Mexico.
3
Department Dermatopathology, Centro Dermatologico "Dr. Ladislao de la Pascua," Ministry of Health of the Government of The Federal District of Mexico City, Mexico.
Abstract
Leprosy has been a challenge in different areas of medicine; in underdeveloped countries it remains a public health problem, in which the social and economic problems facilitate the disease persistence. The diagnosis and consequently the treatment are delayed due to the clinical polymorphism of leprosy, which especially at the beginning the manifestations are not as evident, as is the case of diffuse lepromatous leprosy. This favors the disabilities and the development of the reaction episodes. Fortunately, reaction episodes have decreased with the use of multidrug therapy, and better control of the type 2 reactions has been managed with the use of thalidomide, as in Lucio's phenomenon.
Author information
1
Department of Dermatology, Centro Dermatologico "Dr. Ladislao de la Pascua," Ministry of Health of the Government of The Federal District of Mexico City, Mexico. Electronic address: fermin_santac@yahoo.com.mx.
2
Department of Dermatology, Centro Dermatologico "Dr. Ladislao de la Pascua," Ministry of Health of the Government of The Federal District of Mexico City, Mexico.
3
Department Dermatopathology, Centro Dermatologico "Dr. Ladislao de la Pascua," Ministry of Health of the Government of The Federal District of Mexico City, Mexico.
Abstract
Leprosy has been a challenge in different areas of medicine; in underdeveloped countries it remains a public health problem, in which the social and economic problems facilitate the disease persistence. The diagnosis and consequently the treatment are delayed due to the clinical polymorphism of leprosy, which especially at the beginning the manifestations are not as evident, as is the case of diffuse lepromatous leprosy. This favors the disabilities and the development of the reaction episodes. Fortunately, reaction episodes have decreased with the use of multidrug therapy, and better control of the type 2 reactions has been managed with the use of thalidomide, as in Lucio's phenomenon.
===============================
47.) Lucio's phenomenon: report of five cases.
===============================
47.) Lucio's phenomenon: report of five cases.
===============================
Curi PF1, Villaroel JS2, Migliore N3, Albertengo A4, Aquino ML5, Ceccato
F1, Paira S6.
Author information
1
Rheumatology Section, Hospital JM Cullen, Av Freyre 2150- (3000), Santa Fe, Argentina.
2
Department of Internal Medicine, Hospital Interzonal San Juan Bautista, Catamarca, Argentina.
3
Department of Pathology, Hospital JM Cullen, Santa Fe, Argentina.
4
Department of Pathology, Hospital Iturraspe, Santa Fe, Argentina.
5
Department of Internal Medicine, Sanatorio Chaco SRL, Chaco, Argentina.
6
Rheumatology Section, Hospital JM Cullen, Av Freyre 2150- (3000), Santa Fe, Argentina. pairasergio@fibertel.com.ar.
Abstract
The different clinical forms of leprosy are mainly related to the variety of immunological responses to the infection. Several forms of lepromatous leprosy are recognized, including macular, nodular, and diffuse. Lucio's phenomenon is a rare but distinctive skin eruption seen in patients with diffuse lepromatous leprosy. The diffuse lesions of Lucio's phenomenon have a predilection for the extremities, can include nodules, and heal with atrophic stellate scars; histologically, a necrotizing vasculitis accompanied by a nonspecific inflammatory reaction may be seen. Because of its rarity and similarity with some manifestations of the rheumatic disease and other causes of vasculitis, Lucio's phenomenon may not be easily recognized, especially in non-endemic countries, which leads to confusing diagnosis and loss of time for treatment. We report five patients with vasculitis caused by Lucio's phenomenon.
Author information
1
Rheumatology Section, Hospital JM Cullen, Av Freyre 2150- (3000), Santa Fe, Argentina.
2
Department of Internal Medicine, Hospital Interzonal San Juan Bautista, Catamarca, Argentina.
3
Department of Pathology, Hospital JM Cullen, Santa Fe, Argentina.
4
Department of Pathology, Hospital Iturraspe, Santa Fe, Argentina.
5
Department of Internal Medicine, Sanatorio Chaco SRL, Chaco, Argentina.
6
Rheumatology Section, Hospital JM Cullen, Av Freyre 2150- (3000), Santa Fe, Argentina. pairasergio@fibertel.com.ar.
Abstract
The different clinical forms of leprosy are mainly related to the variety of immunological responses to the infection. Several forms of lepromatous leprosy are recognized, including macular, nodular, and diffuse. Lucio's phenomenon is a rare but distinctive skin eruption seen in patients with diffuse lepromatous leprosy. The diffuse lesions of Lucio's phenomenon have a predilection for the extremities, can include nodules, and heal with atrophic stellate scars; histologically, a necrotizing vasculitis accompanied by a nonspecific inflammatory reaction may be seen. Because of its rarity and similarity with some manifestations of the rheumatic disease and other causes of vasculitis, Lucio's phenomenon may not be easily recognized, especially in non-endemic countries, which leads to confusing diagnosis and loss of time for treatment. We report five patients with vasculitis caused by Lucio's phenomenon.
==================================================
48.) Severe leprosy reactions due to Mycobacterium lepromatosis.
==================================================
48.) Severe leprosy reactions due to Mycobacterium lepromatosis.
==================================================
Han XY1, Jessurun J.
Author information
1
Department of Laboratory Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA. xhan@mdanderson.org
Abstract
Leprosy is caused by the well-known Mycobacterium leprae and the newly discovered M lepromatosis. Here, the authors describe 2 cases of leprosy with unusual clinical presentation caused by M lepromatosis. The patients, a 32-year-old man and a 50-year-old woman, both of Mexican origin, manifested high fever, lymphadenopathy and florid skin lesions in the form of erythema nodosum and Lucio's phenomenon as the first clinical presentation. Heavy infiltration of acid-fast bacilli was identified in the tissues that led to the diagnosis of lepromatous leprosy or diffuse leprosy. The patients were treated with multidrug regimen and responded appropriately. From the lymph node tissue, the authors showed the bacillus to be M lepromatosis, not M leprae as presumed previously, by differential polymerase chain reactions and analysis of gene sequences. These cases add to the growing studies on this organism, expand its endemic regions in Mexico and provide more clinical insight.
Author information
1
Department of Laboratory Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA. xhan@mdanderson.org
Abstract
Leprosy is caused by the well-known Mycobacterium leprae and the newly discovered M lepromatosis. Here, the authors describe 2 cases of leprosy with unusual clinical presentation caused by M lepromatosis. The patients, a 32-year-old man and a 50-year-old woman, both of Mexican origin, manifested high fever, lymphadenopathy and florid skin lesions in the form of erythema nodosum and Lucio's phenomenon as the first clinical presentation. Heavy infiltration of acid-fast bacilli was identified in the tissues that led to the diagnosis of lepromatous leprosy or diffuse leprosy. The patients were treated with multidrug regimen and responded appropriately. From the lymph node tissue, the authors showed the bacillus to be M lepromatosis, not M leprae as presumed previously, by differential polymerase chain reactions and analysis of gene sequences. These cases add to the growing studies on this organism, expand its endemic regions in Mexico and provide more clinical insight.
================================
49.) [Lucio's phenomenon in a patient with leprosy on Aruba].
================================
49.) [Lucio's phenomenon in a patient with leprosy on Aruba].
================================
[Article in Dutch]
Huits RM1, Oskam L, van Raalte JA.
Author information
1
Dr. Horacio E. Oduber Hospitaal, afd. Interne geneeskunde, Oranjestad, Aruba. ralph.huits@gmail.com
Abstract
BACKGROUND:
Lucio's phenomenon is a rare leprosy reaction characterised by bizarrely-shaped, purpuric skin lesions and ulceration. It occurs in diffuse lepromatous leprosy and it is mainly reported in patients from Mexico and the Caribbean.
CASE DESCRIPTION:
We describe the case of a 90-year-old Aruban man with recurrent leg ulcers and flexion contractures of the lower extremities. Occurrence of Lucio's phenomenon led to a diagnosis of diffuse lepromatous leprosy. Presence of Mycobacterium leprae was demonstrated in skin, bone marrow and lymph nodes.
CONCLUSION:
Lucio's phenomenon led to a diagnosis of leprosy. Leprosy is still endemic in Aruba.
Huits RM1, Oskam L, van Raalte JA.
Author information
1
Dr. Horacio E. Oduber Hospitaal, afd. Interne geneeskunde, Oranjestad, Aruba. ralph.huits@gmail.com
Abstract
BACKGROUND:
Lucio's phenomenon is a rare leprosy reaction characterised by bizarrely-shaped, purpuric skin lesions and ulceration. It occurs in diffuse lepromatous leprosy and it is mainly reported in patients from Mexico and the Caribbean.
CASE DESCRIPTION:
We describe the case of a 90-year-old Aruban man with recurrent leg ulcers and flexion contractures of the lower extremities. Occurrence of Lucio's phenomenon led to a diagnosis of diffuse lepromatous leprosy. Presence of Mycobacterium leprae was demonstrated in skin, bone marrow and lymph nodes.
CONCLUSION:
Lucio's phenomenon led to a diagnosis of leprosy. Leprosy is still endemic in Aruba.
================================
50.) Lucio's phenomenon is a necrotizing panvasculitis: mostly a medium-sized granulomatous arteritis.
================================
Am J Dermatopathol. 2008 Dec;30(6):555-60. doi:
10.1097/DAD.0b013e318181270a.
Magaña M1, Fernández-Díez J, Magaña ML.
Author information
1
Centro Médico Nacional Siglo XXI, I.M.S.S., Service of Dermatology, Hospital General de México and School of Medicine, Universidad Nacional Autónoma de México (UNAM), Mexico City, Mexico. dermatopatologia@hotmail.com
Abstract
Lucio's phenomenon (LPh) is a vasculitis clinically described in 1852 and microscopically documented in 1948 in patients with diffuse lepromatous leprosy; however, at present, there is no a clear concept about the pathogenesis of the necrosis, or about the type, size, and site of the damaged vessel. The objective of this study was to elucidate the type, size, site, and form of vessel damage in LPh in a retrospective, clinical, and histopathological study. Clinical information was obtained from the charts and records and/or from the histopathology request. Slides stained with hematoxylin and eosin, Ziehl-Neelsen, and Fite-Faraco were retrieved from our files. Direct immunofluorescence had been performed in 6 cases. Twelve cases fulfilled clinical evidence to make unequivocal diagnosis of diffuse lepromatous leprosy with LPh. All of them had necrotic, irregular, purpuric, and/or ulcerative lesions, which under the microscope showed medium-sized arteries, with their walls involved by clusters of macrophages containing large amounts of bacilli, distortion of the structure of the vessel wall, narrowing, and obliteration of their lumen. Smaller vessels showed changes of the leukocytoclastic type. LPh is a distinctive type of granulomatous and necrotizing panvasculitis; the involved vessels are mostly medium-sized arteries, located deeply in the skin, at the base, and within the hypodermis, but any other vessel is likewise involved, their occlusion leads to ischemic necrosis of the whole skin, frequently with detachment of the epidermis. These changes explain clearly and logically the clinical features observed more than 150 years ago.
Magaña M1, Fernández-Díez J, Magaña ML.
Author information
1
Centro Médico Nacional Siglo XXI, I.M.S.S., Service of Dermatology, Hospital General de México and School of Medicine, Universidad Nacional Autónoma de México (UNAM), Mexico City, Mexico. dermatopatologia@hotmail.com
Abstract
Lucio's phenomenon (LPh) is a vasculitis clinically described in 1852 and microscopically documented in 1948 in patients with diffuse lepromatous leprosy; however, at present, there is no a clear concept about the pathogenesis of the necrosis, or about the type, size, and site of the damaged vessel. The objective of this study was to elucidate the type, size, site, and form of vessel damage in LPh in a retrospective, clinical, and histopathological study. Clinical information was obtained from the charts and records and/or from the histopathology request. Slides stained with hematoxylin and eosin, Ziehl-Neelsen, and Fite-Faraco were retrieved from our files. Direct immunofluorescence had been performed in 6 cases. Twelve cases fulfilled clinical evidence to make unequivocal diagnosis of diffuse lepromatous leprosy with LPh. All of them had necrotic, irregular, purpuric, and/or ulcerative lesions, which under the microscope showed medium-sized arteries, with their walls involved by clusters of macrophages containing large amounts of bacilli, distortion of the structure of the vessel wall, narrowing, and obliteration of their lumen. Smaller vessels showed changes of the leukocytoclastic type. LPh is a distinctive type of granulomatous and necrotizing panvasculitis; the involved vessels are mostly medium-sized arteries, located deeply in the skin, at the base, and within the hypodermis, but any other vessel is likewise involved, their occlusion leads to ischemic necrosis of the whole skin, frequently with detachment of the epidermis. These changes explain clearly and logically the clinical features observed more than 150 years ago.
============================
51.) Livedo reticularis in type 2 lepra reaction: A rare presentation.
============================
51.) Livedo reticularis in type 2 lepra reaction: A rare presentation.
============================
Indian Dermatol Online J. 2014 Apr;5(2):182-4. doi:
10.4103/2229-5178.131097.
Naveen KN1, Athanikar SB1, Hegde SP1, Athanikar VS2.
Author information
1
Department of Dermatology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India.
2
Department of Pathology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India.
Abstract
Type 2 lepra reaction or erythema nodosum leprosum (ENL) is an immune complex syndrome that occurs mostly in lepromatous leprosy and sometimes in borderline lepromatous leprosy patients. We present an untreated case of lepromatous leprosy, who presented with type 2 lepra reaction and livedo reticularis. Livedo reticularis, though seen in lucio phenomenon, is not a part of type 2 lepra reaction. The case is being reported for its rarity.
Naveen KN1, Athanikar SB1, Hegde SP1, Athanikar VS2.
Author information
1
Department of Dermatology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India.
2
Department of Pathology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka, India.
Abstract
Type 2 lepra reaction or erythema nodosum leprosum (ENL) is an immune complex syndrome that occurs mostly in lepromatous leprosy and sometimes in borderline lepromatous leprosy patients. We present an untreated case of lepromatous leprosy, who presented with type 2 lepra reaction and livedo reticularis. Livedo reticularis, though seen in lucio phenomenon, is not a part of type 2 lepra reaction. The case is being reported for its rarity.
============================
52.) Is it Lucio Phenomenon or Necrotic Erythema Nodosum Leprosum?
============================
52.) Is it Lucio Phenomenon or Necrotic Erythema Nodosum Leprosum?
============================
Indian J Dermatol. 2013 Mar;58(2):160. doi: 10.4103/0019-5154.108087.
Ranugha P1, Chandrashekar L, Kumari R, Thappa DM, Badhe B.
Author information
1
Department of Dermatology, Jawaharlal Nehru Institute of Post Graduate Medical Education and Research, Puducherry, India.
Abstract
Lucio phenomenon (LP) or erythema necroticans is a relatively rare, peculiar reaction pattern occurring in untreated lepromatous (LL) or borderline lepromatous (BL) leprosy cases. A 38-year-old male, a cook by occupation, was referred to the dermatology clinic from otolaryngology department with blistering over both the hands and feet of 2 days duration. He had been admitted 1 week back with epistaxis and nasopharyngeal myiasis in otolaryngology department. He was started on systemic antibiotics gentamycin, crystalline penicillin, and metronidazole with nasal instillation of turpentine oil 2 drops 6 times a day. Two days later, he had developed edema with painless hemorrhagic blistering over the dorsum of left hand followed by involvement of the right hand, dorsa of both feet, and both the earlobes within a day. Histopathology of the blister showed sub-epidermal blister, with necrotizing leukocytoclastic vasculitis of papillary dermal vessels with thrombosis, numerous acid-fast bacilli in macrophages, and macrophage granulomas extending up to subcutis. In view of the absent fever or constitutional symptoms, and the classical angular infarcts and hemorrhagic blisters evolving into ulcers with angulated margins, we considered LP as the most likely diagnosis. The patient was started on a combination of WHO recommended multibacillary anti-leprosy therapy and prednisolone (40 mg/day).
Ranugha P1, Chandrashekar L, Kumari R, Thappa DM, Badhe B.
Author information
1
Department of Dermatology, Jawaharlal Nehru Institute of Post Graduate Medical Education and Research, Puducherry, India.
Abstract
Lucio phenomenon (LP) or erythema necroticans is a relatively rare, peculiar reaction pattern occurring in untreated lepromatous (LL) or borderline lepromatous (BL) leprosy cases. A 38-year-old male, a cook by occupation, was referred to the dermatology clinic from otolaryngology department with blistering over both the hands and feet of 2 days duration. He had been admitted 1 week back with epistaxis and nasopharyngeal myiasis in otolaryngology department. He was started on systemic antibiotics gentamycin, crystalline penicillin, and metronidazole with nasal instillation of turpentine oil 2 drops 6 times a day. Two days later, he had developed edema with painless hemorrhagic blistering over the dorsum of left hand followed by involvement of the right hand, dorsa of both feet, and both the earlobes within a day. Histopathology of the blister showed sub-epidermal blister, with necrotizing leukocytoclastic vasculitis of papillary dermal vessels with thrombosis, numerous acid-fast bacilli in macrophages, and macrophage granulomas extending up to subcutis. In view of the absent fever or constitutional symptoms, and the classical angular infarcts and hemorrhagic blisters evolving into ulcers with angulated margins, we considered LP as the most likely diagnosis. The patient was started on a combination of WHO recommended multibacillary anti-leprosy therapy and prednisolone (40 mg/day).
============================
53.) Concomitant lucio phenomenon and erythema nodosum in a leprosy patient: clues for their distinct pathogeneses.
=============================
53.) Concomitant lucio phenomenon and erythema nodosum in a leprosy patient: clues for their distinct pathogeneses.
=============================
Benard G1, Sakai-Valente NY, Bianconcini Trindade MA.
Author information
1
Division of Clinical Dermatology, Clinics Hospital, Medical School of the University of São Paulo, São Paulo, Brazil.
Abstract
Lepromatous leprosy patients may develop necrotic lesions, usually in the context of Lucio phenomenon (LP) or severe erythema nodosum (EN). The clinical and histopathological characteristics of the necrotic manifestations of both entities may eventually be confounded. We describe a patient with lepromatous leprosy who developed, since the 4th month of her first pregnancy, recurrent necrotic lesions in lower limbs, which, at the postpartum, worsened and led to partial destruction of ears and nose. In addition, she referred painful nodes on upper limbs since 1 year before pregnancy and intermittent swelling and tenderness of the ankles, which together with a right tibial and ulnar neuritis led to the diagnosis of, erythema nodosum leprosum (ENL). The histopathology of a biopsy of the upper limb (ENL) revealed a dermal-hypodermal inflammation with vasculitis and vascular lumen narrowing, whereas biopsy of the lower limb (LP) revealed small vessels with fibrin thrombi on the superficial layer of the dermis without inflammatory infiltrate and no evidence of vasculitis. Thus, besides having several different clinical features, LP and ENL result from different pathogenetic mechanisms. The histopathological and clinical features distinguishing both entities are proposed. This distinction is important because decrease in bacillary load through multidrug therapy is the main target in LP, whereas in ENL, concomitant reduction of the reaction by means of thalidomide or high-dose steroids is recommended.
Author information
1
Division of Clinical Dermatology, Clinics Hospital, Medical School of the University of São Paulo, São Paulo, Brazil.
Abstract
Lepromatous leprosy patients may develop necrotic lesions, usually in the context of Lucio phenomenon (LP) or severe erythema nodosum (EN). The clinical and histopathological characteristics of the necrotic manifestations of both entities may eventually be confounded. We describe a patient with lepromatous leprosy who developed, since the 4th month of her first pregnancy, recurrent necrotic lesions in lower limbs, which, at the postpartum, worsened and led to partial destruction of ears and nose. In addition, she referred painful nodes on upper limbs since 1 year before pregnancy and intermittent swelling and tenderness of the ankles, which together with a right tibial and ulnar neuritis led to the diagnosis of, erythema nodosum leprosum (ENL). The histopathology of a biopsy of the upper limb (ENL) revealed a dermal-hypodermal inflammation with vasculitis and vascular lumen narrowing, whereas biopsy of the lower limb (LP) revealed small vessels with fibrin thrombi on the superficial layer of the dermis without inflammatory infiltrate and no evidence of vasculitis. Thus, besides having several different clinical features, LP and ENL result from different pathogenetic mechanisms. The histopathological and clinical features distinguishing both entities are proposed. This distinction is important because decrease in bacillary load through multidrug therapy is the main target in LP, whereas in ENL, concomitant reduction of the reaction by means of thalidomide or high-dose steroids is recommended.
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54.) Lucio's phenomenon. Case study of an exceptional response to treatment exclusively with multibacillary multidrug therapy.
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54.) Lucio's phenomenon. Case study of an exceptional response to treatment exclusively with multibacillary multidrug therapy.
=============================
Peixoto AB1, Portela PS1, Leal FR2, Brotas AM3, Rodrigues NC4.
Author information
1
Marcílio Dias Naval Hospital, Rio de JaneiroRJ, Brazil.
2
Rio de Janeiro Pontifical Catholic University.
3
Rio de Janeiro State University.
4
Marcílio Dias Naval Hospital dermatology clinics, Rio de JaneiroRJ, Brazil.
Abstract
Lucio's phenomenon represents a serious cutaneous necrotizing reaction, which can occur with Lucio's leprosy and also in other forms of lepromatous leprosy. The authors discuss the case of a 63-year-old male patient presenting with ulcers and necrotic cutaneous lesions on the limbs, torso and face, associated with recurring sinusitis, hoarseness and a weight loss of 25 kilos over the last year. Bacilloscopy of intradermal scrapings showed a bacilloscopic index of 5 and the histopathological findings were compatible with the diagnosis of Lucio's phenomenon. The chosen treatment was exclusively multibacillary multidrug therapy, resulting in important dermatological improvement after one month of therapy. The authors present an impressive case of Lucio's phenomenon with an exceptional response to treatment exclusively with multibacillary multidrug therapy.
Author information
1
Marcílio Dias Naval Hospital, Rio de JaneiroRJ, Brazil.
2
Rio de Janeiro Pontifical Catholic University.
3
Rio de Janeiro State University.
4
Marcílio Dias Naval Hospital dermatology clinics, Rio de JaneiroRJ, Brazil.
Abstract
Lucio's phenomenon represents a serious cutaneous necrotizing reaction, which can occur with Lucio's leprosy and also in other forms of lepromatous leprosy. The authors discuss the case of a 63-year-old male patient presenting with ulcers and necrotic cutaneous lesions on the limbs, torso and face, associated with recurring sinusitis, hoarseness and a weight loss of 25 kilos over the last year. Bacilloscopy of intradermal scrapings showed a bacilloscopic index of 5 and the histopathological findings were compatible with the diagnosis of Lucio's phenomenon. The chosen treatment was exclusively multibacillary multidrug therapy, resulting in important dermatological improvement after one month of therapy. The authors present an impressive case of Lucio's phenomenon with an exceptional response to treatment exclusively with multibacillary multidrug therapy.
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55.) [Lucio-Latapí leprosy and the Lucio phenomenon].
==================
55.) [Lucio-Latapí leprosy and the Lucio phenomenon].
==================
Acta Leprol. 1983 Jul-Sep;1(3):115-32.
[Article in French]
Saúl A, Novales J.
Abstract
The Lucio-Latapí's leprosy or diffuse lepromatous leprosy is a clinical variety of lepromatous leprosy first described by Lucio and Alvarado in 1852 and reidentified by Latapí in 1936. It is frequent in Mexico (23%) and in Costa Rica and very rare in other countries. It is characterized by a diffuse infiltration of all the skin which never is transformed into nodule, by a complete alopecia of eyebrows and eyelashes and body hair, by anhydrotic and dysesthesic zones of the skin and by a peculiar type of lepra reaction named Lucio's phenomenon or necrotic erythema which is a vascularitis of vessels especially of the dermohypodermic union and of the hypodermis. Clinically this vascularitis is represented by well-shaped erythematous spots, later becoming necrotic with scabs, ulcerations and scars. Three points of confusion are stressed: the differences between nodules and nudosities, Lucio's leprosy and Lucio's phenomenon and necrotic erythema and necrotic erythema nodosum leprosum. The differences between the pure and primitive form of Lucio's leprosy and the secondary one is also discussed such as the laboratory findings, histopathological data, pronostic and treatment. Lucio's leprosy is considered the most anergic one of the all immunological spectrum of leprosy.
[Article in French]
Saúl A, Novales J.
Abstract
The Lucio-Latapí's leprosy or diffuse lepromatous leprosy is a clinical variety of lepromatous leprosy first described by Lucio and Alvarado in 1852 and reidentified by Latapí in 1936. It is frequent in Mexico (23%) and in Costa Rica and very rare in other countries. It is characterized by a diffuse infiltration of all the skin which never is transformed into nodule, by a complete alopecia of eyebrows and eyelashes and body hair, by anhydrotic and dysesthesic zones of the skin and by a peculiar type of lepra reaction named Lucio's phenomenon or necrotic erythema which is a vascularitis of vessels especially of the dermohypodermic union and of the hypodermis. Clinically this vascularitis is represented by well-shaped erythematous spots, later becoming necrotic with scabs, ulcerations and scars. Three points of confusion are stressed: the differences between nodules and nudosities, Lucio's leprosy and Lucio's phenomenon and necrotic erythema and necrotic erythema nodosum leprosum. The differences between the pure and primitive form of Lucio's leprosy and the secondary one is also discussed such as the laboratory findings, histopathological data, pronostic and treatment. Lucio's leprosy is considered the most anergic one of the all immunological spectrum of leprosy.
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56.) Unusual Presentation of Necrotic Erythema Nodosum Leprosum on Scalp: A Case Report.
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56.) Unusual Presentation of Necrotic Erythema Nodosum Leprosum on Scalp: A Case Report.
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Indian J Lepr. 2015 Jan-Mar;87(1):23-6.
Barman KD, Madan A, Garg VK, Goel K, Khurana N.
Abstract
Lepra reactions are acute episodes occurring during the disease process of leprosy and are of 2 types: type 1 or reversal reaction and type 2 reaction or erythema odosumleprosum (ENL). In the episodes of lepra reaction several parts are affected including face and extremities like oral cavity. In the present case report we reported a rare case of lepromatous leprosy with necrotic ENL involving scalp apart from the usual sites. A 58 year old married male presented to us with complaints of spontaneous onset, recurrent eruption of multiple reddish raised painful lesions. Biopsy from the infiltrated skin over the back showed atrophic epidermis, free Grenz zone, diffuse and periadnexal macrophage granulomas with predominant mononuclear infiltrate, appandageal atrophy, fibrosis around the neural structures and leukocytoclastic vasculitis. Fites stain showed strong positivity for M. leprae. His routine blood investigations showed anemia (Hb = 7.8 gm%), neutrophil leukocytosis (TLC = 17,600, DLC = P66L28M4E2) and raised ESR (80 mm in the first hour). These bullous and necrotic lesions in leprosy may be a manifestation of severe type II reactions in patients with very high bacillary load.
Barman KD, Madan A, Garg VK, Goel K, Khurana N.
Abstract
Lepra reactions are acute episodes occurring during the disease process of leprosy and are of 2 types: type 1 or reversal reaction and type 2 reaction or erythema odosumleprosum (ENL). In the episodes of lepra reaction several parts are affected including face and extremities like oral cavity. In the present case report we reported a rare case of lepromatous leprosy with necrotic ENL involving scalp apart from the usual sites. A 58 year old married male presented to us with complaints of spontaneous onset, recurrent eruption of multiple reddish raised painful lesions. Biopsy from the infiltrated skin over the back showed atrophic epidermis, free Grenz zone, diffuse and periadnexal macrophage granulomas with predominant mononuclear infiltrate, appandageal atrophy, fibrosis around the neural structures and leukocytoclastic vasculitis. Fites stain showed strong positivity for M. leprae. His routine blood investigations showed anemia (Hb = 7.8 gm%), neutrophil leukocytosis (TLC = 17,600, DLC = P66L28M4E2) and raised ESR (80 mm in the first hour). These bullous and necrotic lesions in leprosy may be a manifestation of severe type II reactions in patients with very high bacillary load.
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57.) Lucio's phenomenon: another case reported in Brazil.
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57.) Lucio's phenomenon: another case reported in Brazil.
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An Bras Dermatol. 2012 Mar-Apr;87(2):296-300.
Monteiro R1, Abreu MA, Tiezzi MG, Roncada EV, Oliveira CC, Ortigosa LC.
Author information
1
Dermatology Service, Hospital Regional de Presidente Prudente, Universidade do Oeste Paulista, Presidente Prudente, SP, Brazil. rodrigomt@hotmail.com
Abstract
Lucio's phenomenon is defined as a variant of type 2 leprosy reaction. It is a rare event, occurring in the evolution of leprosy of Lucio and other forms of lepromatous leprosy. It has an exacerbated proliferation of Hansen bacilli in its pathophysiology, which invade blood vessel walls and injure endothelial cells, causing endothelial proliferation and decreasing the vascular lumen. This fact, associated with inflammatory reactions and changes in the coagulation system causes vascular thrombosis, ischemia, infarction and tissue necrosis, leading to the histopathological characteristic of the phenomenon. We report a case of lepromatous leprosy with irregular treatment that developed Lucio's phenomenon. Treatment with multidrug therapy, antibiotics, steroids and thalidomide achieved a favorable outcome.
Monteiro R1, Abreu MA, Tiezzi MG, Roncada EV, Oliveira CC, Ortigosa LC.
Author information
1
Dermatology Service, Hospital Regional de Presidente Prudente, Universidade do Oeste Paulista, Presidente Prudente, SP, Brazil. rodrigomt@hotmail.com
Abstract
Lucio's phenomenon is defined as a variant of type 2 leprosy reaction. It is a rare event, occurring in the evolution of leprosy of Lucio and other forms of lepromatous leprosy. It has an exacerbated proliferation of Hansen bacilli in its pathophysiology, which invade blood vessel walls and injure endothelial cells, causing endothelial proliferation and decreasing the vascular lumen. This fact, associated with inflammatory reactions and changes in the coagulation system causes vascular thrombosis, ischemia, infarction and tissue necrosis, leading to the histopathological characteristic of the phenomenon. We report a case of lepromatous leprosy with irregular treatment that developed Lucio's phenomenon. Treatment with multidrug therapy, antibiotics, steroids and thalidomide achieved a favorable outcome.
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