The Pityriasis Lichenoides, a paraneoplastic syndrome?
The Pityriasis Lichenoides, a paraneoplastic syndrome?
La Pitiriasis liquenoide, un síndrome paraneoplasico?
EDITORIAL ESPAÑOL:
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Hola amigos de la red, esta patología, la PITIRIASIS LIQUENOIDE, la publique en el año 1999, la actualización la hice un año después (2000), donde hago una ampliación de la información sobre ella, con mas referencias bibliográficas, y hoy 2025 hago una nueva actualización de la misma, con mas información, los ÚLTIMOS Y CLÁSICOS TRATAMIENTOS, con nuevas referencias.
HISTORIA:
Los primeros en describir la PITIRIASIS LIQUENOIDE fueron Neisser y Jadassohn en 1894, describiéndola como una enfermedad idiopática y poco común, que afecta tanto a niños como adultos con dos variantes: LA PITIRIASIS LIQUENOIDE AGUDA, y LA PITIRIASIS LIQUENOIDE CRÓNICA.
Posteriormente en el año de 1.916 MUCHA describió por primera vez la forma AGUDA de esta enfermedad y en 1925 HABERMANN también hizo lo mismo, siendo desde entonces conocida con el nombre de 1.) PITIRIASIS LIQUENOIDE VARIOLIFORME AGUDA DE MUCHA HABERMANN. (PLEVA).
También se describió la forma CRÓNICA de la enfermedad con el nombre de 2.) PITIRIASIS LIQUENOIDE CRÓNICA (PLC).
En el año de 1.996 DEGOS describió una forma febril y ULCERO NECRÓTICA de la clásica enfermedad de MUCHA HABERMAN (FUMH), conocida y aceptada en el círculo científico como 3.) PITIRIASIS LIQUENOIDE ULCERONECRÓTICA, pero se le atribuyó su nombre a los científicos MUCHA Y HABERMAN, con las siglas FUMHD:
F=FEBRIL
U=ULCERONECRÓTICA
M=MUCHA
H=HABERMANN
D=DISEASE.
La cual, como dije en la revisión previa, es la variante aparatosa, de la PITIRIASIS LIQUENOIDE AGUDA (PLEVA), caracterizada por un brote papuloso que evoluciona a ampollas y necrosis, acompañado de fiebre intensa, y malestar general.
También hay descrita una 4ta variante por algunos autores denominada 4.) PITIRIASIS LIQUENOIDE MELANODERMICA, que como también explicamos es la versión de la PITIRIASIS LIQUENOIDE AGUDA o CRÓNICA que deja MANCHAS HIPOCRÓMICAS RESIDUALES, sobre todo la (PLC).
Enfermedad de difícil tratamiento y comportamiento incierto, de causa desconocida, se ha asociado en algunas ocasiones como un estado previo a malignidad (linfoma), también siendo asociada a la papulosis linfomatoide la cual a su vez también ha sido relacionada con linfomas cutáneos (CTCL)
En esta revisión bibliográfica encontré algunos aspectos interesantes que resalto a continuación:
1.) EXISTEN 3 VARIANTES DE LA ENFERMEDAD, RECONOCIDOS ACTUALMENTE: (2025)
A.) PITIRIASIS LIQUENOIDE VARIOLIFORME AGUDA 1.916- 1925-(PLEVA).
La Aguda tiene una variante: B.) LA PITIRIASIS LIQUENOIDE ULCERONECRÓTICA (forma febril) de MUCHA HABERMANN (1.966 (Degos) (FUMH)
C.) PITIRIASIS LIQUENOIDE CRÓNICA. (PLC).
Un dato importantísimo en esta enfermedad, es que la PITIRIASIS LIQUENOIDE CRÓNICA, puede aparecer como CRÓNICA de inicio, no necesariamente tiene que evolucionar de la AGUDA a la CRÓNICA, como ocurre con muchas enfermedades.
DATOS RELEVANTES DE LAS VARIANTES:
1.) La 4ta Variante no está reconocida dentro de la clasificación: son solo 3: PLEVA, PLC y FUMHD.
2.) Ambas variantes han sido asociada a estados malignos (linfoma).
3.) Se pueden presentar tanto en niños como adultos.
4.) Se ha observado una mayor asociación con malignidad en los adultos que en niños.
5.) Se han descrito casos de remisión después de tonsilectomía.
6.) Se han descrito casos en una misma familia.
7.) Recientemente se describieron en febrero del 2.000 tres casos en niños donde se encontró en las biopsias cambios histopatológicos compatibles con micosis fungoides.
8.)También se han descrito casos de pitiriasis liquenoide crónica, que se transformaron en parakeratosis variegata. (parapsoriasis).
9.) La forma aguda puede desaparecer o evolucionar hacia la forma crónica o micosis fungoides
10.) la forma crónica puede aparecer como crónica por primera vez, desaparecer o evolucionar a papulosis linfomatoide o linfoma.
11.) Entre las posibles causas etiológicas se menciona un posible estado de hiperreactividad a un agente infeccioso, entre ellos el virus de Epstein Barr, Toxoplasma Gondii e infección estreptocócica.
12.) En un estudio hecho en Egipto en 1.997 sobre 22 pacientes con PLC se consiguió que el 36.36% tenia toxoplasmosis.
13.) También ha sido descrita en asociación a enfermedades reumáticas: artritis reumatoide.
14.)Se han encontrado depósitos de IGM y C3 en biopsias de pacientes con PITIRIASIS LIQUENOIDE AGUDA Y CRÓNICA. Una enfermedad por complejos inmunes ?
15.) No hay tratamiento específico pero las tetraciclinas, eritromicina, metotrexato, pentoxifilina, tacrolimus, pimecrolimus, y luz ultravioleta son las mas usados.
NUEVAS TERAPIAS:
El DUPILUMAB: es un anticuerpo monoclonal inhibidor de las interleuquinas IL-4 y IL-13, DOS CITOQUINAS claves que disminuyen la inflamación, desarrollado por el laboratorio SANOFI-AVENTIS, con el nombre de DUPIXENT, y aprobado para ser usado en la dermatitis atopica principalmente. Se ha utilizado en la PITIRIASIS LIQUENOIDE con resultados variables, pudiendo empeorarla en algunos casos, de modo que su uso en esta patología no es seguro.
TRATAMIENTOS CLÁSICOS: La AZITROMIZINA, ERITROMICINA, ERITROMICINA, Y MINOCICLINA, han demostrado ser útiles en esta patología, junto con fototerapia, corticosteroides orales y topicos.
En base a estos datos interesantes y EVIDENTES podríamos considerar a la pitiriasis liquenoide y sus variantes como un SÍNDROME muy cercano a la PARA-NEOPLASIA el cual debemos vigilar siempre por su posible evolución a malignidad,
En los exámenes de rutina, pedir siempre títulos de Toxoplasma, Epstein Barr Virus (EBV), y exámenes inmunológicos: C3-Inmunoglobulinas (IGM), etc.
En este enlace encuentras la previa revisión de la PITIRIASIS LIQUENOIDE I
En las 65 referencias los hechos....
Saludos a todos,,,
Dr. José Lapenta R.
EDITORIAL ESPAÑOL
==================
Hello friends of the internet, I made this update a year later (2000), where I expanded the information on this pathology, which I later updated in 2017.
HISTORY:
The first to describe pityriasis lichenoides were Neisser and Jadassohn in 1894, describing it as a rare, idiopathic disease that affects both children and adults with two variants: pityriasis lichenoides acuta and pityriasis lichenoides chronica.
Later, in 1916, MUCHA, described the acute form of this disease for the first time, and in 1925, HABERMANN did the same. Since then, it has been known as 1.) PITYRIASIS LICHENOIDES ET VARIOLIFORMIS ACUTE of MUCHA HABERMANN (PLEVA).
The chronic form of the disease was also described under the name 2.) PITYRIASIS LICHENOIDES CHRONICA (PLC).
In 1996, Degos described a febrile and ulcerative necrotic form of the classic MUCHA-HABERMANN disease (FUMHD), known and accepted in scientific circles as 3.) PITYRIASIS LICHENOIDES FEBRILE AND ULCERONECROTIC. However, its name was attributed to the scientists MUCHA and HABERMAN, with the acronym FUMHD:
F = FEBRILE
U = ULCERONECROTIC
M = MUCHA
H = HABERMANN
D = DISEASE.
This, as I mentioned in the previous review, is the stricken form of PITYRIASIS LICHENOIDES ACUTE (PLEVA), characterized by a papular rash that progresses to blisters and necrosis, accompanied by high fever and general malaise.
A fourth variant has also been described by some authors, called 4.) PITYRIASIS LICHENOIDES MELANODERMIC: which, as we also explained, is the version of acute or chronic pityriasis lichenoides that leaves residual hypochromic spots, especially PLC.
A disease of difficult treatment and uncertain behavior, with an unknown cause, it has sometimes been associated with a pre-malignancy (lymphoma), and has also been associated with lymphomatoid papulosis, which in turn has also been linked to cutaneous lymphomas (CTCL).
In this literature review, I found some interesting aspects that I highlight below:
1.) THERE ARE 3 CURRENTLY RECOGNIZED VARIANTS OF THE DISEASE: (2025)
A.) PITYRIASIS LICHENOIDES VARIOLIFORMIS ACUTE 1916-1925 (PLEVA).
The acute form has one variant: B.) PITYRIASIS LICHENOIDES ULCERONECROTIC (febrile form) of MUCHA HABERMANN (1966, Degos, (FUMH)
C.) PITYRIASIS LICHENOIDES CHRONICA (PLC).
Very important fact about this disease is that PITYRIASIS LICHENOIDES CHRONICA can appear CHRONIC from the onset; it does not necessarily have to progress from ACUTE to CHRONIC, as occurs with many diseases.
RELEVANT DATA ABOUT THE VARIANTS:
1.) The 4th Variant is not recognized within the classification: there are only 3: PLEVA, PLC and FUMHD.
2.) Both variants have been associated with malignant conditions (lymphoma).
3.) They can occur in both children and adults.
4.) A greater association with malignancy has been observed in adults than in children.
5.) Cases of remission after tonsillectomy have been described.
6.) Cases have been described in the same family.
7.) Three cases were recently described in February 2000 in children where histopathological changes compatible with mycosis fungoides were found in biopsies.
8.) Cases of pityriasis lichenoides chronica, which transformed into parakeratosis variegata (parapsoriasis), have also been described.
9.) The acute form may disappear or evolve into the chronic form or mycosis fungoides.
10.) The chronic form may appear chronic for the first time, disappear, or evolve into lymphomatoid papulosis or lymphoma.
11.) Possible etiological causes include a possible state of hyperreactivity to an infectious agent, including the Epstein-Barr virus, Toxoplasma gondii, and streptococcal infection.
12.) In a 1997 study conducted in Egypt on 22 patients with PLC, 36.36% were found to have toxoplasmosis.
13.) It has also been described in association with rheumatic diseases: rheumatoid arthritis.
14.) IGM and C3 deposits have been found in biopsies of patients with Pityriasis Lichenoides Acute and Chronica. Is this an immune complex disease?
15.) There is no specific treatment, but tetracyclines, erythromycin, methotrexate, pentoxifylline, tacrolimus, pimecrolimus, and ultraviolet light are the most commonly used.
NEW THERAPIES:
DUPILUMAB is a
monoclonal antibody that inhibits the interleukins IL-4 and IL-13, two key
cytokines that reduce inflammation. It was developed by SANOFI-AVENTIS
under the name DUPIXENT and is
approved primarily for use in atopic dermatitis. It has been used in
pityriasis lichenoides with variable results, and can worsen the condition
in some cases, so its use in this condition is not safe.
TRADITIONAL TREATMENTS:
Azithromycin, erythromycin, and minocycline have been shown to be useful
in this condition, along with phototherapy and oral and topical
corticosteroids.
Based on these interesting and EVIDENT data, we could consider pityriasis lichenoides and its variants a syndrome very similar to PARANEOPLASIA, which we should always monitor for possible malignancy.
In routine examinations, always request titers for Toxoplasma, Epstein-Barr Virus (EBV), and immunological tests: C3-Immunoglobulins (IGM), etc.
At this link, you can find the previous review of PITYRIASIS LICHENOIDES I.
In the 65 references, the facts...
Greetings to all
Dr. José Lapenta.
Saludos a Todos. !!!
Dr. Jose Lapenta.
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REFERENCIAS BIBLIOGRAFICAS /
BIBLIOGRAPHICAL REFERENCES
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A.- A Systematic Review of Treatments for Pityriasis Lichenoides (2019).
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1.) Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association
with pityriasis lichenoides et varioliformis
2.) Lymphomatoid papulosis/pityriasis lichenoides in two
children.
3.) Clinical and histologic features of pityriasis Lichenoides et
varioliformis acuta in children.
4.) Pityriasis lichenoides in children: therapeutic response to
erythromycin.
5.) Pityriasis lichenoides in children: a long-term follow-up of
eighty-nine cases.
6.) Clinical and histologic differentiation between lymphomatoid
papulosis and pityriasis lichenoides.
7.) Phototherapy of pityriasis lichenoides.
8.) [Pityriasis lichenoides in a sibling pair]
9.) Febrile ulceronecrotic Mucha-Habermann's disease.
10.) Pityriasis lichenoides chronica resolving after tonsillectomy
[letter]
11.) Pityriasis lichenoides and lymphomatoid papulosis.
12.) Lymphomatoid papulosis: clinicopathological comparative study
with pityriasis lichenoides et varioliformis acuta.
13.)Comparative clinicopathological study on pityriasis lichenoides
chronica and small plaque parapsoriasis.
14.) Severe febrile Mucha-Habermann's disease in children: case
report and review of the literature.
15.) The histologic spectrum of mycosis fungoides/Sezary syndrome
(cutaneous T-cell lymphoma). A review of 222 biopsies, including newly
described patterns and the earliest pathologic changes.
16.) UV-B phototherapy for pityriasis lichenoides.
17.) [Parakertosis variegata after pityriasis lichenoides et
varioliformis acuta]
18.) Febrile ulceronecrotic pityriasis lichenoides et
varioliformis acuta.
19.) Febrile ulceronecrotic pityriasis lichenoides et
varioliformis acuta.
20.) [Pityriasis-lichenoides-et-varioliformis-acuta-like drug
exanthema caused by astemizole]
21.) Atypical manifestations of pityriasis lichenoides chronica:
development into paraneoplasia and non-Hodgkin lymphomas of the skin.
22.) Pityriasis lichenoides-like eruption occurring during therapy
for myelogenous leukemia.
23.) Immunopathologic studies in pityriasis lichenoides.
24.)Immunohistology of pityriasis lichenoides et varioliformis
acuta and pityriasis lichenoides chronica. Evidence for their
interrelationship with lymphomatoid papulosis.
25.) Clonal T-cell populations in pityriasis lichenoides et
varioliformis acuta (Mucha-Habermann disease).
26.) Immunopathology of pityriasis lichenoides acuta.
27.)Psoralens and ultraviolet A therapy of pityriasis lichenoides.
28.) Histopathologic diagnosis of pityriasis lichenoides et
varioliformis acuta and its clinical correlation.
29.) Long-term follow-up of photochemotherapy in pityriasis
lichenoides.
30.) Pityriasis lichenoides, an immune complex disease?
31.) [Pityriasis lichenoides (author's transl)]
32.) HIV seropositivity in association with pityriasis
33.) Koebnerization as a cutaneous manifestation of immune
complex-mediated vasculitis.
34.) Pentoxifylline (Trental) therapy for vasculitis of pityriasis
lichenoides et varioliformis [letter]
35.) Lymphomatoid papulosis and pityriasis lichenoides: are they
related?
36.) Immunofluorescence findings in pityriasis lichenoides
37.) Febrile ulceronecrotic Mucha-Habermann disease.
38.) Immunohistochemical distinction of lymphomatoid papulosis and
pityriasis lichenoides et varioliformis acuta.
39.) Benign and neoplastic eosinophilic staining cells: an
immunofluorescence study.
40.) Differentiation and clonality of lesional lymphocytes in
small plaque parapsoriasis [see comments]
41.) Examination of cutaneous T-cell lymphoma for human
herpesviruses by using the polymerase chain reaction.
42.) Mucha-Habermann disease in a child: possible association with
measles vaccination.
43.) Mucha-Habermann disease in children -- the association with
rheumatic diseases.
44.) [cutaneous and neurologic vasculitis disclosing EBV-selective
immunodeficiency].
45. [Lichenoid pityriasis (parapsoriasis guttata) in children.
Report of 17 cases].
46.) [Lichenoid pityriasis. Immunologic study of 10 children].
47.) Febrile ulceronecrotic Mucha-Habermann's disease with
interstitial pneumonitis.
48.) Lichenoid pityriasis. Clinical study of 13 cases].
49.) The transformation of pityriasis lichenoides chronica into
parakeratosis variegata in an 11-year-old girl.
50.) Mucha-Habermann disease and its febrile ulceronecrotic
variant.
51.)Febrile ulceronecrotic Mucha-Habermann disease.
52.) Mucha-Habermann disease: a diagnostic possibility for
prolonged fever associated with systemic and skin symptoms.
53.) [Acute parapsoriasis in a 5-year-old girl].
54.) Mucha-Habermann's disease and arthritis: possible association
with reactivated Epstein-Barr virus infection.
55.) [Mucha-Habermann disease. Description of a case in
childhood].
56.) Mucha-Habermann disease in children -- the association with
rheumatic diseases.
57.) Mucha-Habermann's disease in children: treatment with
erythromycin.
58.) Histiocytic medullary reticulosis presenting as
Mucha-Habermann disease.
59.) Methotrexate for the treatment of Mucha-Habermann disease.
60.)Pityriasis lichenoides-like mycosis fungoides in children.
61.) Pityriasis lichenoides in children: clinicopathologic review
of 22 patients.
62.) The relation between toxoplasmosis and pityriasis lichenoides
chronica.
63.) Experience with UVB phototherapy in children.
64.) Pityriasis lichenoides of childhood with atypical
CD30-positive cells and clonal T-cell receptor gene rearrangements.
65.) Pityriasis lichenoides et varioliformis acuta and group-A
beta hemolytic streptococcal infection.
66.) Treatment of adult diffuse pityriasis lichenoides chronica
with narrowband ultraviolet B: experience and literature review.
67.) Phototherapy in children: Considerations and indications.
68.) Phototherapy for Pityriasis Lichenoides in the Pediatric
Population: A Review of the Published Literature.
69.) [Febrile ulceronecrotic Mucha-Habermann disease].
70.) Pityriasis Lichenoides in Childhood: Review of Clinical
Presentation and Treatment Options.
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1.) Cutaneous T-cell lymphoma (parapsoriasis en plaque). An
association with pityriasis lichenoides et varioliformis
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acuta in young children.
SO - Arch Dermatol 1990 Nov;126(11):1449-53
AU - Fortson JS; Schroeter AL; Esterly NB
PT - JOURNAL ARTICLE
AB - Pityriasis lichenoides et varioliformis acuta (PLEVA) and
pityriasis lichenoides chronica (PLC) are related benign disorders
without recognized association with cutaneous T-cell lymphoma (CTCL). We
report the cases of two children with documented PLEVA evolving into
CTCL over several years. One child had the clinical lesions of PLC but
the dermatopathologic findings of PLEVA at age 2 years. At age 12 years,
he had skin changes of poikiloderma atrophicans vasculare and
dermatopathologic findings consistent with parapsoriasis en plaque. The
second child presented at age 7 years with scaling dermatitis and
dermatopathologic findings of PLEVA. At age 12 years, the histologic
diagnosis was parapsoriasis. Monoclonal antibody studies performed on
biopsy specimens from both patients revealed 70% to 100% cells staining
with CD5, 80% to 90% staining with CD4, 30% to 50% staining with CD8,
and an increase in CD1-staining cells in the papillary dermis,
indicating a predominantly helper T-cell infiltrate. We believe that PLC
and PLEVA may be part of the spectrum of CTCL. Furthermore, CTCL may be
more common in young children than once thought.
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2.) Lymphomatoid papulosis/pityriasis lichenoides in two children.
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SO - Pediatr Dermatol 1987 Nov;4(3):238-41
AU - Ashworth J; Paterson WD; MacKie RM
PT - JOURNAL ARTICLE
AB - Two children developed lymphomatoid papulosis/pityriasis
lichenoides at ages 3 and 6 years. Follow-up continued for 13 years in
the former patient and for 6 years in the latter. Both children now have
continuing low-grade disease activity requiring in the one case topical
corticosteroid therapy and in the other low-dose systemic steroid
therapy. These children are reported to emphasize to pediatricians,
pediatric pathologists, and hematologists that pseudolymphomatous
conditions can exist in young children and do not require potent
cytotoxic therapy. In both of our patients, the initial diagnosis was
thought to be an aggressive lymphoma.
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3.) Clinical and histologic features of pityriasis Lichenoides et
varioliformis acuta in children.
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SO - Arch Dermatol 1987 Oct;123(10):1335-9
AU - Longley J; Demar L; Feinstein RP; Miller RL; Silvers DN
PT - JOURNAL ARTICLE
AB - Pityriasis lichenoides et varioliformis acuta (PLEVA) is
commonly thought of as a disease of young adults, yet we identified five
cases, involving patients who were 3, 5, 6, 8, and 11 years of age,
among 13,000 consecutive specimens submitted to a general
dermatopathology laboratory during a 15-week period. The clinical and
histologic features of PLEVA in our cases were similar to those reported
for adults, except that no lesions were observed on the scalp or mucous
membranes of children. A high index of suspicion and biopsy specimens of
suspected lesions are often needed to differentiate PLEVA from other
papular and crusted eruptions seen in the pediatric age group. These
include reactions to arthropods, Gianotti-Crosti syndrome, varicella,
and erythema multiforme. Histologically, papular eczema and pityriasis
rosea may be misdiagnosed as PLEVA.
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4.) Pityriasis lichenoides in children: therapeutic response to
erythromycin.
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SO - J Am Acad Dermatol 1986 Jul;15(1):66-70
AU - Truhan AP; Hebert AA; Esterly NB
PT - JOURNAL ARTICLE
AB - Fifteen of twenty-two children with pityriasis lichenoides
were treated with oral erythromycin. Eleven (73%) had a remission,
usually within 2 months. Two others showed partial improvement, and two
were unimproved. Seven of the children who experienced a remission were
off erythromycin and free of lesions after 2 to 5 months of therapy. A
trial of erythromycin as described herein should be considered in
children with pityriasis lichenoides before other, possibly more toxic,
measures are instituted.
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5.) Pityriasis lichenoides in children: a long-term follow-up of
eighty-nine cases.
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SO - J Am Acad Dermatol 1990 Sep;23(3 Pt 1):473-8
AU - Gelmetti C; Rigoni C; Alessi E; Ermacora E; Berti E; Caputo R
PT - JOURNAL ARTICLE
AB - Pityriasis lichenoides is usually classified into an acute
and a chronic form. From a review of 89 cases of the disease seen since
1974 it seems that a more realistic classification into three main
groups, according to the distribution of pityriasis lichenoides lesions,
could be made, namely, a diffuse, a central, and a peripheral form, each
characterized by a different clinical course. Conversely, no
correlations were detected in our series between the severity of skin
lesions and their distribution or the overall course of the disease.
None of our cases suggests the possible evolution of pityriasis
lichenoides into lymphomatoid papulosis. Although no infectious
causative agent has been identified, a viral origin seems likely in some
cases. Most patients responded favorably to
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irradiation. Our conclusions are (1) that pityriasis lichenoides
is probably a clinical disorder with a diverse etiology and (2) that its
classification by distribution seems more useful than its subdivision
into an acute and a chronic form.
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6.) Clinical and histologic differentiation between lymphomatoid
papulosis and pityriasis lichenoides.
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SO - J Am Acad Dermatol 1985 Sep;13(3):418-28
AU - Willemze R; Scheffer E
PT - JOURNAL ARTICLE
AB - The relationship between lymphomatoid papulosis and
pityriasis lichenoides is a matter of considerable debate.
Differentiation between these two conditions is, however, important
because patients with lymphomatoid papulosis, unlike those with
pityriasis lichenoides, may develop systemic lymphoma and thus require
long-term follow-up. In our study the clinical and histologic features
of eighty-two patients with pityriasis lichenoides and twenty-six
patients with lymphomatoid papulosis were reviewed and compared.
Clinical and histologic differences were recognized, not only allowing
differentiation between the two conditions, but also suggesting that
they are pathogenetically distinct diseases. Finally, evidence is
presented to suggest that the different views on the relationship
between these diseases mainly result from differences in patient
selection.
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7.) Phototherapy of pityriasis lichenoides.
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SO - Arch Dermatol 1983 May;119(5):378-80
AU - Le Vine MJ
PT - JOURNAL ARTICLE
AB - Eleven patients with chronic pityriasis lichenoides chronica
were treated with topically applied bland emollient cream and minimally
erthemogenic doses of UV radiation from fluorescent sunlamps. The
conditions of all patients cleared completely in an average of 29
treatments, requiring an average UV dose of 388 millijoules/sq cm at
clearance. Phototherapy provides a convenient effective outpatient
therapy for pityriasis lichenoides chronica.
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8.) [Pityriasis lichenoides in a sibling pair]
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SO - Hautarzt 1981 Nov;32(11):592-4
AU - Deuchert C
PT - JOURNAL ARTICLE
AB - Two brothers are reported, who had pityriasis lichenoides
within an interval of eighteen months. The hitherto unknown etiology of
this dermatosis is discussed.
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9.) Febrile ulceronecrotic Mucha-Habermann's disease.
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SO - J Dermatol 1994 Jan;21(1):46-9
AU - Maekawa Y; Nakamura T; Nogami R
PT - JOURNAL ARTICLE; REVIEW (9 references); REVIEW OF REPORTED
CASES
AB - Febrile ulceronecrotic Mucha-Habermann's disease (FUMH) was
first described by Degos in 1966. In the literature, nine cases of FUMH
have been reported in both children and adults. We report a 16-year-old
boy with the febrile ulceronecrotic type. A review of the nine cases in
the literature showed acute necrotic lesions, as well as rare
complications such as fever, superinfected lesions and viral infection
which are not as common in pityriasis lichenoides et varioliformis
acuta. There is no definitive treatment, but systemic corticosteroid,
methotrexate, antibiotics (tetracycline, erythromycin), aciclovir, and
4,4-diaminodiphenyl sulfone (DDS) have been frequently used. The most
common histologic feature is mononuclear perivascular infiltrates
consisting of T lymphocytes. The etiology is not known, but a
hypersensitivity reaction, possibly to an infectious agent, is
suggested.
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10.) Pityriasis lichenoides chronica resolving after tonsillectomy
[letter]
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SO - Br J Dermatol 1993 Sep;129(3):353-4
AU - Takahashi K; Atsumi M
PT - LETTER
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11.) Pityriasis lichenoides and lymphomatoid papulosis.
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SO - Semin Dermatol 1992 Mar;11(1):73-9
AU - Rogers M
PT - JOURNAL ARTICLE; REVIEW (79 references); REVIEW, TUTORIAL
AB - The clinical features, histopathology, immunopathology, and
management of pityriasis lichenoides and lymphomatoid papulosis are
discussed, with particular emphasis on the pediatric aspects of these
conditions. The difficulties in logically separating pityriasis
lichenoides into an acute (pityriasis lichenoides et varioliformis
acuta) and a chronic (pityriasis lichenoides chronical) form are
addressed. The development of lymphoreticular malignancy in patients
with lymphomatoid papulosis has been well documented, but pityriasis
lichenoides has characteristically been regarded as a benign condition.
However, recent reports of the development of large plaque parapsoriasis
in patients with pityriasis lichenoides have led to a reconsideration.
Some of these patients were in the pediatric age group. Although there
are significant clinical, histopathological, and immunopathological
differences between pityriasis lichenoides and lymphomatoid papulosis,
the demonstration of similar clonal T cell receptor gene rearrangements
and the confirmation of the potentially premalignant nature of both
suggests that there may indeed be an interrelationship between these two
controversial entities. Close follow-up of patients with both of these
conditions is recommended, with observation being discontinued only when
the patient has been free of lesions for several years.
=============================================================
12.) Lymphomatoid papulosis: clinicopathological comparative study
with pityriasis lichenoides et varioliformis acuta.
=============================================================
SO - J Dermatol 1991 Oct;18(10):580-5
AU - Erpaiboon P; Mihara I; Niimura M
PT - JOURNAL ARTICLE
AB - We have compared the clinical and histopathological features
of 6 patients with lymphomatoid papulosis (LP) and 14 patients with
pityriasis lichenoides et varioliformis acuta (PLEVA). There were some
differences between the clinical features in the two diseases, including
the size and appearance of skin lesions and the duration of the course
of disease. Ki-1 Ag positive, large, atypical, lymphoid cells were
always seen in lymphomatoid papulosis; none of lymphoid cells of
pityriasis lichenoides et varioliformis acuta demonstrated this antigen.
We conclude that lymphomatoid papulosis and PLEVA, although sharing some
common features, should be considered to be different clinical and
immunopathological entities.
=============================================================
13.)Comparative clinicopathological study on pityriasis
lichenoides chronica and small plaque parapsoriasis.
=============================================================
SO - Am J Dermatopathol 1988 Jun;10(3):189-96
AU - Benmaman O; Sanchez JL
PT - JOURNAL ARTICLE
AB - The term parapsoriasis refers to a group of chronic
asymptomatic scaly dermatoses of unknown etiology about which there is
still controversy over the nosology and nomenclature of the different
conditions that comprise the group, particularly pityriasis lichenoides
chronica (PLC) and small plaque parapsoriasis (SPP). In an attempt to
establish the distinctive clinicopathologic features of these two
dermatosis, we prospectively studied 44 patients who presented with the
typical clinical and histologic picture of either of these two diseases.
SPP was clinically characterized by scaly oval plaques on the trunk and
proximal aspect of extremities. Spongiosis was the salient
histopathologic feature, with absence of fibrosis or melanophages. PLC
presented with a scaly papular eruption over the trunk and extremities
and histologically was characterized by an interface dermatitis. We
conclude that sufficient clinical and histologic features differentiate
these two entities and we propose that the term parapsoriasis be used
only to designate SPP and large plaque parapsoriasis.
============================================================
14.) Severe febrile Mucha-Habermann's disease in children: case
report and review of the literature.
=============================================================
SO - Pediatr Dermatol 1991 Mar;8(1):51-7
AU - Luberti AA; Rabinowitz LG; Ververeli KO
PT - JOURNAL ARTICLE; REVIEW (25 references); REVIEW OF REPORTED
CASES
AB - Mucha-Habermann disease, or pityriasis lichenoides et
varioliformis acuta, is usually a benign, papulosquamous, cutaneous
disorder. It has also been reported in a severe form with fever and
systemic symptoms both in children and adults. We report a 12-year-old
boy with the febrile, ulceronecrotic type. A review of similar cases in
the literature shows a 16% frequency of acute necrotic lesions, as well
as rare complications such as fever, superinfected lesions, bacteremia
(most often with Staphylococcus aureus), and rheumatologic
manifestations such as arthritis and scleroderma. There is no definitive
treatment, but tetracycline, erythromycin, methotrexate, and ultraviolet
light are used most frequently. The most common histologic feature is
mononuclear perivascular infiltrates. Mucha-Habermann disease can mimic
other common entities such as varicella and insect bites.
=============================================================
15.) The histologic spectrum of mycosis fungoides/Sezary syndrome
(cutaneous T-cell lymphoma). A review of 222 biopsies, including newly
described patterns and the earliest pathologic changes.
=============================================================
SO - Am J Surg Pathol 1994 Jul;18(7):645-67
AU - Shapiro PE; Pinto FJ
PT - JOURNAL ARTICLE
AB - We studied 222 skin biopsies of mycosis fungoides and Sezary
syndrome (cutaneous T-cell lymphoma [CTCL]) to document the huge
histologic spectrum and to evaluate the earliest histologic changes. Our
results indicate that CTCL produces practically all of the patterns used
for diagnosing inflammatory skin disease: superficial or superficial and
deep perivascular without epidermal changes; spongiotic; psoriasiform,
with or without a lichenoid infiltrate; interface, including lichenoid
without vacuolar alteration, lichenoid with vacuolar alteration, and
vacuolar alteration without a lichenoid infiltrate; follicular, with or
without mucin; nodular and diffuse; vasculitis; vesicular; and
panniculitis. Unusual examples resembling granuloma annulare, gyrate
erythema, lichen planus, and pityriasis lichenoides were seen. To
further document the spectrum within each pattern, we analyzed many
variables, such as lymphocytic atypia, epidermotropism, epidermal
contour, and composition of the dermal infiltrate. Common clues to the
diagnosis of CTCL include epitheliotropism with little spongiosis;
lymphocytes lined up along the basal layer; hyperconvoluted lymphocytes;
and broad areas of slight hyperorthokeratosis that is compact or
laminated, with subtle interspersed parakeratosis. Less common clues
include Pautrier's microabscesses; granulomatous foci; coexistence of
plasma cells and eosinophils; and rounded, hyperplastic rete ridges
adjacent to flattened rete. The earliest changes of CTCL appear to be a
sparse, superficial perivascular infiltrate with slight or no epidermal
hyperplasia and with rare lymphocytes in the lower epidermis, especially
the basal layer, often with hyperconvoluted nuclei. Our findings support
the hypothesis that CTCL develops sui generis, rather than from another
chronic dermatosis.
=============================================================
16.) UV-B phototherapy for pityriasis lichenoides.
=============================================================
SO - Australas J Dermatol 1985 Apr;26(1):9-13
AU - Siew NT
PT - JOURNAL ARTICLE
=============================================================
=============================================================
17.) [Parakertosis variegata after pityriasis lichenoides et
varioliformis acuta]
=============================================================
SO - Hautarzt 1995 Jul;46(7):498-501
AU - Kiene P; Folster-Holst R; Mielke V
PT - JOURNAL ARTICLE
AB - We report on a 34-year-old male patient who developed
generalized parakeratosis variegata lesions 4 years after suffering from
pityriasis lichenoides et varioliformis acuta. For further investigation
of a possible interrelationship between these two diseases of the
parapsoriasis group and their relationship to the T-cell type of
cutaneous non-Hodgkin-lymphoma, histological, immunohistological and
molecular-biological techniques were applied. We were able to
demonstrate typical morphological features common to both diseases, and
a polyclonal T-cell infiltrate in both. It is concluded that pityriasis
lichenoides et varioliformis acuta and parakeratosis variegata are
separate entities without monoclonal rearrangement or signs of
malignancy.
=============================================================
18.) Febrile ulceronecrotic pityriasis lichenoides et
varioliformis acuta.
=============================================================
SO - J Am Acad Dermatol 1994 Feb;30(2 Pt 1):261-3
AU - Fink-Puches R; Soyer HP; Kerl H
PT - JOURNAL ARTICLE; REVIEW (10 references); REVIEW OF REPORTED
CASES
=============================================================
=============================================================
19.) Febrile ulceronecrotic pityriasis lichenoides et
varioliformis acuta.
=============================================================
SO - Dermatology 1994;189 Suppl 2:50-3
AU - De Cuyper C; Hindryckx P; Deroo N
PT - JOURNAL ARTICLE
AB - An unusually severe form of pityriasis lichenoides et
varioliformis acuta (PLEVA) with a fatal outcome in an 82-year-old woman
is reported. After a period of a mild eruption, extensive polymorphous,
papular and ulcerohemorrhagic skin lesions developed, associated with
intermittent high temperature and constitutional symptoms. Skin biopsies
showed the typical histopathological changes of PLEVA. Early recognition
of this severe variant of PLEVA is important, since the fulminating
course can lead to death.
=============================================================
20.) [Pityriasis-lichenoides-et-varioliformis-acuta-like drug
exanthema caused by astemizole]
=============================================================
SO - Hautarzt 1993 Apr;44(4):235-7
AU - Stosiek N; Peters KP; von den Driesch P
PT - JOURNAL ARTICLE
AB - We report on a 40-year-old male patient who developed an
unusual generalized drug eruption taking the form of a histologically
confirmed pityriasis lichenoides et varioliformis acuta (PLEVA) after
oral intake of the H1-antagonist astemizole. On two occasions,
independently repeated medication with astemizole exacerbated the
typical rash again. Oral exposure and the specific lymphocyte
transformation test confirmed the suspected causal connection between
astemizole and PLEVA.
=============================================================
21.) Atypical manifestations of pityriasis lichenoides chronica:
development into paraneoplasia and non-Hodgkin lymphomas of the skin.
=============================================================
SO - Dermatology 1992;184(1):65-9
AU - Panizzon RG; Speich R; Dazzi H
PT - JOURNAL ARTICLE
AB - Three patients with atypical courses and manifestations of
pityriasis lichenoides chronica (PLC) are presented. The first patient
is a 21-year-old white woman who showed a good response of her PLC
lesions as well as her reactive oligoarthritis to repeated PUVA
treatments combined with oral prednisone during 1 year. The effect of
the treatment then decreased. The patient developed a low-grade
malignant lymphoma of the lung. When the lymphoma of the lung improved
after chemotherapy, the PLC eruptions improved, too. The second patient
is a 41-year-old man, whose Hodgkin's disease stage IVa was successfully
treated by chemotherapy and radiotherapy in 1984. In 1987 he showed PLC
lesions which responded well to PUVA therapy, later also in combination
with etretinate. Until 1988 repeated skin biopsies revealed a
non-specific eczematous pattern. In 1989 the recalcitrant PLC eruptions
finally revealed a pleomorphic non-Hodgkin lymphoma of the skin with
medium-sized cells. The third patient had a PLC for about 9 years when
Hodgkin's disease stage Ia was diagnosed. At the beginning the skin
biopsy showed an eczematous pattern, but 2 years later, in 1990, skin
infiltrations of a large-cell, anaplastic non-Hodgkin lymphoma were
seen. These cases show that PLC in rare cases may either represent a
paraneoplastic skin disease or may itself develop into cutaneous
lymphomas.
=============================================================
22.) Pityriasis lichenoides-like eruption occurring during therapy
for myelogenous leukemia.
=============================================================
SO - J Dermatol 1989 Feb;16(1):73-5
AU - Isoda M
PT - JOURNAL ARTICLE
AB - A 61-year-old Japanese man with chronic myelogenous leukemia
developed pityriasis lichenoides-like eruptions during chemotherapy.
Histopathological features were also consistent with the disease. The
eruption in this case may have been an allergic reaction arising in a
depressed immunity induced by chemotherapy.
=============================================================
23.) Immunopathologic studies in pityriasis lichenoides.
=============================================================
SO - Arch Dermatol Res 1988;280 Suppl:S61-5
AU - Giannetti A; Girolomoni G; Pincelli C; Benassi L
PT - JOURNAL ARTICLE
AB - Skin biopsy specimens from five patients with pityriasis
lichenoides et varioliformis acuta and from six patients with pityriasis
lichenoides chronica were studied by direct immunofluorescence and by an
immunoperoxidase technique using a panel of monoclonal antibodies. The
dermal inflammatory infiltrate was composed of T cells, macrophages, and
a small proportion of CD1a+ cells, mostly perivascular. CD8+ cells
(cytotoxic/suppressor phenotype) predominated in the epidermis according
to the degree of epidermal necroses, whereas CD4+ cells (helper/inducer
phenotype) were superior in number among dermal T cells. A few B cells
and Leu7+ cells were detected in only a small proportion of lesions. The
results obtained confirm that the two conditions are variants of a
single disease process and suggest that cell-mediated immune mechanisms
may be important in the pathogenesis of the epidermal and vascular
damage. Endothelial cells (HLA-DR+ and HLA-DQ+) and CD1a+ cells
(epidermal and possibly dermal) could be primarily involved, acting as
antigen-presenting cells.
=============================================================
24.)Immunohistology of pityriasis lichenoides et varioliformis
acuta and pityriasis lichenoides chronica. Evidence for their
interrelationship with lymphomatoid papulosis.
=============================================================
SO - J Am Acad Dermatol 1987 Mar;16(3 Pt 1):559-70
AU - Wood GS; Strickler JG; Abel EA; Deneau DG; Warnke RA
PT - JOURNAL ARTICLE
AB - Pityriasis lichenoides et varioliformis acuta and pityriasis
lichenoides chronica are idiopathic, papular eruptions that exhibit
certain clinicopathologic similarities to each other and to lymphomatoid
papulosis. In order to determine if these disorders are also similar
immunologically, we studied the immunopathology of five biopsy specimens
from three cases of pityriasis lichenoides et varioliformis acuta and
three biopsy specimens from three cases of pityriasis lichenoides
chronica. We then compared them to our prior immunohistologic study of
nine cases of lymphomatoid papulosis. Pityriasis lichenoides et
varioliformis acuta and pityriasis lichenoides chronica both exhibited a
dermal and epidermal infiltrate of CD4+ and CD8+ T cells expressing
activation antigens. These were admixed with numerous macrophages. The
lesional epidermis was diffusely human lymphocyte antigen (HLA)-DR+ and
contained decreased CD1+ dendritic cells. Endothelial cells were also
HLA-DR+. Cells bearing the phenotypes of B cells, follicular dendritic
cells, or natural killer/killer cells were essentially absent. Except
for the lack of large atypical cells, the results resembled those
described previously for lymphomatoid papulosis. These findings indicate
that pityriasis lichenoides chronica, pityriasis lichenoides et
varioliformis acuta, and lymphomatoid papulosis share several
immunohistologic features. Together with certain clinicopathologic
similarities, they are consistent with the hypothesis that these three
disorders are interrelated.
=============================================================
25.) Clonal T-cell populations in pityriasis lichenoides et
varioliformis acuta (Mucha-Habermann disease).
=============================================================
SO - Am J Pathol 1987 Mar;126(3):417-21
AU - Weiss LM; Wood GS; Ellisen LW; Reynolds TC; Sklar J
PT - JOURNAL ARTICLE
AB - Patients with the skin disorder pityriasis lichenoides et
varioliformis acuta (PLEVA) develop recurrent, self-healing
papulonecrotic lesions that contain infiltrates of cytologically and
antigenically normal T lymphocytes. DNA extracted from the lesions of 3
patients with PLEVA was analyzed for rearrangement of beta-T-cell
receptor genes for the purpose of assessing the clonality of T
lymphocytes within the tissues of this disease. Lesions from all 3 cases
showed clonal gene rearrangements. In each of 2 cases from which two
separate lesions were biopsied, identical rearrangements were found in
specimens from both sites. DNA from a variety of inflammatory lesions
obtained from patients with other types of skin diseases failed to show
detectable rearrangements of beta-T-cell receptor genes. These results
suggest that PLEVA represents a T-cell lymphoproliferative process,
rather than an inflammatory disorder, as had been previously thought.
=============================================================
26.) Immunopathology of pityriasis lichenoides acuta.
=============================================================
SO - J Am Acad Dermatol 1984 May;10(5 Pt 1):783-95
AU - Muhlbauer JE; Bhan AK; Harrist TJ; Moscicki RA; Rand R;
Caughman W; Loss B; Mihm MC Jr
PT - JOURNAL ARTICLE
AB - Eleven biopsy specimens (five papules and six dusky or
crusted lesions) from four patients with pityriasis lichenoides et
varioliformis acuta ( PLEVA ) were studied by direct immunofluorescence
and immunoperoxidase technics. Slight vascular deposits of IgM and C3
were present in most lesions. Slight perivascular deposits of fibrin
were observed in early lesions; more extensive perivascular and
interstitial deposits of fibrin were detected in advanced lesions. Most
of the infiltrating cells were T lymphocytes; cells with
cytotoxic/suppressor phenotype (T8-positive) were generally more
numerous than cells with helper/inducer phenotype (Leu-3a-positive,
T4-positive). A marked increase in epidermal T8-positive cells over
epidermal Leu-3a/T4-positive cells was found in late lesions. Moreover,
a reduction of the ratio of circulating T4-positive to T8-positive cells
was observed in most cases. The number of epidermal T6-positive
(Langerhans/indeterminate) cells was decreased in the lower as compared
with the upper stratum spinosum. About 5% of perivascular infiltrating
cells were T6-positive. These results suggest that cell-mediated immune
mechanisms are probably important in the pathogenesis of PLEVA
=============================================================
27.)Psoralens and ultraviolet A therapy of pityriasis lichenoides.
=============================================================
SO - J Am Acad Dermatol 1984 Jan;10(1):59-64
AU - Powell FC; Muller SA
PT - JOURNAL ARTICLE
AB - Three patients with long-standing pityriasis lichenoides,
which was resistant to other forms of therapy, were successfully treated
with PUVA (psoralens and ultraviolet light of wavelength A). One patient
had complete clearing of all lesions, and the other two had marked
improvement. PUVA is being used to treat increasing numbers of patients
with pityriasis lichenoides, and the results have been very good.
=============================================================
28.) Histopathologic diagnosis of pityriasis lichenoides et
varioliformis acuta and its clinical correlation.
=============================================================
SO - Arch Dermatol 1982 Jul;118(7):478-82
AU - Hood AF; Mark EJ
PT - JOURNAL ARTICLE
AB - To assess the specificity of the histopathologic features in
the diagnosis of pityriasis lichenoides et varioliformis acuta (PLEVA),
we reviewed the clinical manifestations and courses of 42 patients for
whom this diagnosis was suggested in the pathology report. The
histologic diagnosis of PLEVA was clinically substantiated in 16 of
these 42 cases. Of the 26 cases in which PLEVA was erroneously diagnosed
histologically, the correct clinical diagnosis was suggested before
biopsies were done in 21 instances. In the five remaining cases, both
the prebiopsy clinical diagnosis and the pathologic diagnosis proved to
be incorrect. Pityriasis rosea, insect bites, and eczematous dermatitis
accounted for the majority of the cases that histologically mimicked
PLEVA. The constellation of histologic findings described in PLEVA
(presence of intraepidermal lymphocytes and erythrocytes, dermal
hemorrhage, and so-called lymphocytic vasculitis) is not specific and
may be seen in a variety of dermatologic disorders.
=============================================================
29.) Long-term follow-up of photochemotherapy in pityriasis
lichenoides.
=============================================================
SO - Acta Derm Venereol 1982;62(5):442-4
AU - Boelen RE; Faber WR; Lambers JC; Cormane RH
PT - JOURNAL ARTICLE
AB - Five patients with a histopathologically confirmed diagnosis
of pityriasis lichenoides were treated with PUVA or irradiated with a
light source emitting UVB and UVA, without prior intake of psoralens.
All patients showed a good response to treatment. Long-term follow up
showed that patients remained free of lesions during a period of 20 to
36 months; 3 patients had a recurrence of the disease, though less
extensive than before, after 25, 23, and 23 months, respectively.
=============================================================
30.) Pityriasis lichenoides, an immune complex disease?
=============================================================
SO - Acta Derm Venereol 1980;60(3):259-61
AU - Faber WR; van Joost T
PT - JOURNAL ARTICLE
AB - Nine biopsies from skin lesions of 5 patients with pityriasis
lichenoides acuta and three biopsies from skin lesions of 3 patients
with pityriasis lichenoides chronica were examined by means of the
direct immunofluorescence technique. IgM deposits along the
dermoepidermal junction were found in only two biopsies. In the majority
of bioipsies, complement (C3) deposits were found along the
dermo-epidermal junction and in the vessel walls. Immunoglobulin and C3
deposits were not found concomitantly in the vessel walls.
=============================================================
31.) [Pityriasis lichenoides (author's transl)]
=============================================================
SO - Ann Dermatol Venereol 1980;107(10):895-9
AU - Franc MP; Barrut D; Moulin G
PT - JOURNAL ARTICLE; REVIEW (20 references)
AB - A review of the literature concerning the pityriasis
lichenoides and the study of 34 personal cases show that three main
clinical patterns are found in pityriasis lichenoides: maculo-papular,
leukomelanodermal, necrotic. The course is very variable: rarely seven
weeks, more often seven months and sometimes seven years. The disease is
issued from an angiitis including a mostly lymphocytic infiltration. The
epidermis is secondarily invaded by inflammatory cells and shows focal
parakeratosis. There is no specific immunologic disorder:
immunohistopathologic study is generally normal (rarely IgM or C3
deposits); no circulating immune complex is found. Some patients
improved with dapsone or photochemotherapy.
=============================================================
32.) HIV seropositivity in association with pityriasis
============================================================
lichenoides et varioliformis acuta.
SO - Clin Exp Dermatol 1992 Jan;17(1):36-7
AU - Ostlere LS; Langtry JA; Branfoot AC; Staughton RC
PT - JOURNAL ARTICLE
AB - We describe a case of PLEVA in an asymptomatic, human
immunodeficiency virus (HIV) positive patient. This association has not
been previously described. The possible mechanisms involved are
discussed.
=============================================================
33.) Koebnerization as a cutaneous manifestation of immune
complex-mediated vasculitis.
=============================================================
SO - J Am Acad Dermatol 1990 May;22(5 Pt 1):775-81
AU - Chan LS; Cooper KD; Rasmussen JE
PT - JOURNAL ARTICLE
AB - Two unusual examples of the cutaneous manifestations of
vasculitis are presented. In both cases lesions occurred on previously
traumatized skin and on normal skin of the dependent areas. Lesional
skin biopsy specimens obtained from the koebnerized sites and from the
other dependent sites revealed evidence of vascular injury in both
patients. A diagnosis of leukocytoclastic vasculitis was made in one
patient and pityriasis lichenoides et varioliformis acuta in the other.
Direct immunofluorescence microscopy of lesional skin specimens from
both patients demonstrated dermal vascular immune deposits. Raji cell
assay detected a significant elevation of circulating immune complexes
in the serum of both patients. Neither koebnerizing leukocytoclastic
vasculitis nor koebnerizing pityriasis lichenoides et varioliformis
acuta has been reported previously.
=============================================================
34.) Pentoxifylline (Trental) therapy for vasculitis of pityriasis
lichenoides et varioliformis [letter]
=============================================================
SO - Arch Dermatol 1985 Dec;121(12):1487
AU - Sauer GC
PT - LETTER
=============================================================
=============================================================
35.) Lymphomatoid papulosis and pityriasis lichenoides: are they
related?
=============================================================
SO - Br J Dermatol 1982 Jun;106(6):717-21
AU - Black MM
PT - JOURNAL ARTICLE
=============================================================
=============================================================
36.) Immunofluorescence findings in pityriasis lichenoides
[letter]
SO - Br J Dermatol 1980 Jul;103(1):120-1
AU - Nieboer C; Kalsbeek GL
PT - LETTER
=============================================================
=============================================================
37.) Febrile ulceronecrotic Mucha-Habermann disease.
=============================================================
SO - J Am Acad Dermatol 1993 Nov;29(5 Pt 2):903-6
AU - Lopez-Estebaranz JL; Vanaclocha F; Gil R; Garcia B; Iglesias
L
PT - JOURNAL ARTICLE; REVIEW (11 references); REVIEW OF REPORTED
CASES
AB - Febrile ulceronecrotic Mucha-Habermann disease in an
18-year-old man is reported. This disease is a severe form of pityriasis
lichenoides et varioliformis acuta (PLEVA) and is characterized by the
sudden onset of diffuse coalescent ulcerations associated with high
fever and systemic symptoms. In the present case the disease was
preceded by typical PLEVA. Histologically, a leukocytoclastic vasculitis
was seen in addition to the usual features of PLEVA. Findings of
laboratory studies revealed an elevated erythrocyte sedimentation rate,
a high white blood cell count, and a mild increase in liver enzymes. No
systemic involvement was detected. Findings of T cell receptor gene
analysis in skin and peripheral blood showed no abnormality. The patient
was treated with PUVA and methotrexate with a good response. We review
the eight previously reported cases of febrile ulceronecrotic
Mucha-Habermann disease.
=============================================================
38.) Immunohistochemical distinction of lymphomatoid papulosis and
pityriasis lichenoides et varioliformis acuta.
=============================================================
SO - Am J Pathol 1990 Apr;136(4):979-87
AU - Varga FJ; Vonderheid EC; Olbricht SM; Kadin ME
PT - JOURNAL ARTICLE
AB - Lymphomatoid papulosis (LyP) and pityriasis lichenoides et
varioliformis acuta (PLEVA) are benign self-healing cutaneous eruptions
that may be clinically and histologically similar. However LyP has a 5%
to 20% risk of associated lymphoid malignancy, whereas PLEVA does not.
To determine whether the immunophenotype of lymphoid cells is useful in
the distinction of these two disorders, the pattern of expression of
lymphoid cell lineage and activation antigens in nine cases of LyP and
seven cases of PLEVA were compared. In all cases of LyP most larger
cells expressed the activation antigen Ki-1 (CD30) and lacked expression
of the T-cell antigen CD7 and at least one other T-cell antigen (CD2,
CD3, CD5). In contrast, CD30-antigen expression was rare or absent in
PLEVA, CD3- and CD7-antigen expression was found in all cases, and
diminished expression of T-cell antigens (CD2 and CD5) was seen in only
one case. Diffuse expression of HLA-DR antigen by epidermal
keratinocytes was found in a greater proportion of PLEVA cases (6 of 7)
than LyP cases (3 of 6). In addition, CD8+ cells predominated at the
dermal/epidermal junction in 3 of 6 cases of PLEVA but in only 1 of 7
cases of LyP. We conclude that LyP and PLEVA can be distinguished
immunohistochemically in most, if not all, cases. Furthermore these
results suggest that LyP and PLEVA are separate disorders, thus
accounting for their variable prognoses.
=============================================================
39.) Benign and neoplastic eosinophilic staining cells: an
immunofluorescence study.
=============================================================
SO - Br J Dermatol 1980 Feb;102(2):155-60
AU - Danno K; Imamura S; Horio T; Ofuji S
PT - JOURNAL ARTICLE
AB - Deposition of immunoglobulins, complement and fibrinogen on
eosinophilic staining cells was investigated using direct
immunofluorescence techniques. Serum factor deposition was detected on
benign epidermal eosinophilic cells seen in pityriasis lichenoides et
varioliformis acuta, sunburn erythema and, in addition, on subepidermal
hyaline bodies in lichen planus; no such deposition occurred on
neoplastic eosinophilic cells in Bowen's disease and squamous cell
carcinoma. The qualitative findings of immunofluorescence microscopy
seem to be different in inflammatory and malignant dermatoses.
=============================================================
40.) Differentiation and clonality of lesional lymphocytes in
small plaque parapsoriasis [see comments]
=============================================================
SO - Arch Dermatol 1995 Mar;131(3):321-4
AU - Haeffner AC; Smoller BR; Zepter K; Wood GS
PT - JOURNAL ARTICLE
AB - BACKGROUND: Small plaque parapsoriasis is an idiopathic
chronic dermatosis characterized by patches on the trunk and extremities
that are often smaller than 5 cm in diameter and that sometimes have a
digitate contour. These latter cases are often referred to as digitate
dermatosis. Histopathologic examination reveals a mild superficial
perivascular lymphocytic infiltrate associated with mild spongiosis and
parakeratosis. To characterize this disease more completely, we analyzed
the differentiation and clonality of lesional lymphocytes using
immunohistologic and molecular biologic methods. OBSERVATIONS: We
studied five cases using a frozen-section immunoperoxidase technique. In
each case, there was a predominantly CD4+ T-cell infiltrate admixed with
CD8+ T cells, Langerhans cells/indeterminate cells, and macrophages. In
three cases, the clonality of lesional T cells was studied by denaturing
gradient gel electrophoresis of polymerase chain reaction-amplified
T-cell receptor-gamma gene rearrangements. Two cases showed a dominant
clonal pattern, while one case exhibited a polyclonal pattern. Clinical
follow-up disclosed persistent disease in one of the two clonal cases,
while lesions in the other clonal case and the polyclonal case gradually
resolved. CONCLUSIONS: Our findings indicate that small plaque
parapsoriasis is a clinically indolent, histopathologically nonspecific,
predominantly CD4+ T-cell-mediated disease that, at least in some cases,
contains a dominant T-cell clone. These features put small plaque
parapsoriasis into a category with certain other members of the
parapsoriasis group, namely, pityriasis lichenoides and lymphomatoid
papulosis, which have been shown to be clonal T-cell disorders despite
their clinically benign course. It remains to be determined if the
dominant T-cell clones identified in some cases of small plaque
parapsoriasis can ever be the direct precursors of overt cutaneous
T-cell lymphomas.
=============================================================
41.) Examination of cutaneous T-cell lymphoma for human
herpesviruses by using the polymerase chain reaction.
=============================================================
SO - J Cutan Pathol 1993 Aug;20(4):304-7
AU - Brice SL; Jester JD; Friednash M; Golitz LE; Leahy MA;
Stockert SS; Weston WL
PT - JOURNAL ARTICLE
AB - The etiology of cutaneous T-cell lymphoma remains unknown,
although an association with viral infection, in particular certain
retroviruses and human herpesviruses, has been suggested. The purpose of
this study was to examine skin biopsies of cutaneous T-cell lymphoma for
the presence of Epstein-Barr virus, herpes simplex virus type 1 and type
2, and human herpesvirus-6 by using the polymerase chain reaction.
Lesional skin biopsies from 30 patients with cutaneous T-cell lymphoma
were studied. Control specimens included biopsies from 9 patients with
lymphomatoid papulosis and 10 patients with pityriasis lichenoides et
varioliformis acuta. DNA extracted from each specimen, as well as from a
known positive control for each virus, was examined by using the
polymerase chain reaction with viral-specific primers. Each DNA specimen
was also amplified with control primers for human beta globin. The
specificity of the amplified products was confirmed by Southern
analysis. Neither Epstein-Barr virus nor herpes simplex virus was
detected in any of the patient specimens examined. Human herpesvirus-6
was detected in one specimen of cutaneous T-cell lymphoma and one
specimen of lymphomatoid papulosis. These results do not support a role
for any of these herpesviruses in the pathogenesis of cutaneous T-cell
lymphoma.
=============================================================
42.) Mucha-Habermann disease in a child: possible association with
measles vaccination.
=============================================================
SO - J Dermatol 1992 Apr;19(4):253-5
AU - Torinuki W
PT - JOURNAL ARTICLE
AB - A 2.5-year-old boy presented with skin lesions consistent
with Mucha-Habermann disease, which appeared about 5 days after an
injection of freeze-dried live attenuated measles vaccine. He responded
to both oral and topical corticosteroid therapy. To my knowledge, this
represents the first such association of Mucha-Habermann disease with
virus vaccination.
=============================================================
43.) Mucha-Habermann disease in children -- the association with
rheumatic diseases.
=============================================================
SO - J Rheumatol 1982 Mar-Apr;9(2):319-24
AU - Ellsworth JE; Cassidy JT; Ragsdale CG; Sullivan DB
PT - JOURNAL ARTICLE
AB - Two children are described who developed Mucha-Habermann
disease as infants. One boy had juvenile rheumatoid arthritis that ran a
progressive course over 10 years, although his skin disease responded to
a low dose of corticosteroids. One girl had polyarthritis associated
with onset of her rash but both resolved over several years without
treatment. She has since developed scleroderma followed by a
reappearance of her skin lesions.
=============================================================
44.) [cutaneous and neurologic vasculitis disclosing EBV-selective
immunodeficiency].
=============================================================
Ann Dermatol Venereol 1996;123(6-7):387-92 Related Articles,
Books, LinkOut
Grosieux C, Amoric JC, Mechinaud F, Moreau A, Mussini JM, Fesneau
H, Dreno B, Bureau B, Stalder JF, Litoux P
CHU de Nantes, Hotel-Dieu.
INTRODUCTION: Purtilo's syndrome or X-linked lymphoproliferative
syndrome (XLP) is a rare genetic disorder affecting boys who have a
selective immunodeficit towards Epstein Barr Virus (EBV) and who develop
extremely severe forms of EBV infection, of which there are four major
types: severe or fatal infectious mononucleosis (60 p. 100), lymphoma
(23 p. 100), acquired hypo- or agamaglobulinemia (25 p. 100) and anemia
or pancytopenia. We report a case of vasculitis (cutaneous and
neurologic) which led to the discovery of a selective immunodeficit
towards EBV, similar to Purtilo's syndrome. CASE REPORT: A 17 year-old
male with no significant past medical history presented with an eruption
initially felt to be consistent with pityriasis lichenoid. Treatment
with erythromycin was initiated, this did not prevent the subsequent
eruptions of cutaneous vasculitis lesions which were severe, prolonged,
debilitating, and associated with fever and general deterioration of the
patient condition. All etiologic studies were negative. A course of
systemic corticosteroids was begun, but the cutaneous eruptions
persisted; and in addition the patient developed signs of polyneuropathy
in the lower extremities secondary to neurologic vasculitic lesions. New
studies revealed an abnormal EBV serology (absence of anti-EBNA
antibodies) as well as hypogammaglobulinemia, suggestive of a selective
immunodeficit towards EBV resembling Purtilo's syndrome. DISCUSSION: In
our patient, the development of an extensive vasculitis, characterized
histologically by an intense lymphocytic infiltrate, positive for EBV,
associated with hypogammaglobulinemia, and with abnormal serology
suggests an anomaly in the immune response to EBV. Although the age of
the patient and absence of family history make the Purtilo's syndrome
uncertain, the nature of the immunodeficit is very similar and the
patient could well develop a lymphoma. This case is significant in that
the disease initially manifested itself as a cutaneous vasculitis, which
was not been described previously.
=============================================================
45. [Lichenoid pityriasis (parapsoriasis guttata) in children.
Report of 17 cases].
=============================================================
Ann Pediatr (Paris) 1991 Sep;38(7):469-75 Related Articles, Books,
LinkOut
Klene C, Cony M, Plantin P, Sanciaume C, Legrain V, Taieb A,
Maleville J
Service de Dermatologie Pediatrique, Cours de l'Argonne, Bordeaux.
Seventeen cases of pityriasis lichenoides diagnosed over a
nine-year period in children under 15 years of age are reported.
Patients with this benign disease develop papular skin lesions covered
with thick, coherent scales which detach in a single piece (reminiscent
of sealing wax). Pruritis is not marked. Lesions may be necrotic (Mucha
Habermann's small pox-like form, n = 6) or mild (leukodermic form, n =
2). Half of the patients studied developed several episodes and total
duration of the disease exceeded two years in one third of cases.
Recovery occurred after one or two episodes in half the children. Scars
developed in some patients with severely necrotic lesions. None of the
patients developed lymphoma. All patients with lymphomatoid papulosis
progressing to lymphoma reported in the literature were adults.
Pathogenesis of pityriasis lichenoides remains unknown but may involve
lymphocytic vasculitis. No truly effective therapy is available.
However, oral macrolides can be used especially in patients with early
manifestations suggesting an infectious disease. Emollients,
heliotherapy and ultraviolet therapy may also be recommended.
=============================================================
46.) [Lichenoid pityriasis. Immunologic study of 10 children].
=============================================================
Med Cutan Ibero Lat Am 1988;16(3):251-3 Related Articles, Books,
LinkOut
[Article in Spanish]
Gelmetti A, Cerri D, Cebrian Blazquez M
Departamento de Dermatologia I y Dermatologia Pediatrica, Facultad
de Medicina de Milan.
Ten children clinically and histologically diagnosed as having
pityriasis lichenoides (PL), have been studied by direct
immunofluorescence (DIF). Circulating immune complexes (CI) have also
been studied in four children. Granular deposits of IgM, located in the
walls of the dermal vessels have been observed in two cases, but they
have never been found at the dermo-epidermal junction. Granular deposits
of C3 have been observed in three children, both in the walls of the
dermal vessels and at the dermo-epidermal junction. The search for
immune complexes gave negative results in all cases. The hypothesis of
some authors that PL is an immune complex disease cannot be confirmed by
our findings.
=============================================================
47.) Febrile ulceronecrotic Mucha-Habermann's disease with
interstitial pneumonitis.
=============================================================
J Cutan Pathol 1979 Feb;6(1):66-76 Related Articles, Books,
LinkOut
Auster BI, Santa Cruz DJ, Eisen AZ
A case of febrile ulceronecrotic Mucha-Habermann's is presented.
This disorder is a severe form of pityriasis lichenoides et
varioliformis acuta (PLEVA) characterized by the sudden eruption of
diffuse coalescent ulcerations associated with high fever. In the
present case the disease was preceded by the milder typical form of
PLEVA. Histologically a leukocytoclastic vasculitis was seen in addition
to the usual lymphocytic perivascular and lichenoid infiltrate. During
the course of the disease the patient developed an interstitial
pneumonitis which resolved concomitantly with the cutaneous lesions.
Adenovirus type II recovered at the height of the illness from the
patient's urine may have etiologic implications in the pathogenesis of
the disease.
=============================================================
48.) Lichenoid pityriasis. Clinical study of 13 cases].
=============================================================
Med Cutan Ibero Lat Am 1977;5(3):189-96 Related Articles, Books,
LinkOut
Bravo Piris J
13 patients with Pityriasis Lichenoides are studied clinical and
histologically, showing a clinical polymorphism of the lesions, mainly
in the papulous, vesiculous, and necrotic ones. The data about age, sex,
evolution and response to the treatment in the present study are similar
to those found by other authors. Constantly, we found, a variable degree
of vasculitis. In almost all the cases there was a damage of the
epithelium --exoserosis and exocytosis--, as well as presence in some
cases, of red cells extravasated within the epidermis. In upper dermis
we found in all biopsies, divers degrees of perivascular cell
infiltration mainly composed of lymphocytes and histiocytes with
predominance of the last ones, in five cases. In the majority of our
cases, there was a strong relationship between the clinical and the
histological aspects, but in some cases, mild lesions showed an acute
microscopical picture. We are of the opinion that Pityriasis Lichenoides
must be considered as a different entity from Parapsoriasis. In
addition, we think that PL, is a clinical picture that manifests itself
as a chronic or an acute form, and both types can be seen in the disease
evolution. Finally, we could not find an evident influence and a
positive response to the treatment in our patients with the classical
therapeutics.
=============================================================
49.) The transformation of pityriasis lichenoides chronica into
parakeratosis variegata in an 11-year-old girl.
=============================================================
Br J Dermatol 1997 Dec;137(6):983-7 Related Articles, Books,
LinkOut
Niemczyk UM, Zollner TM, Wolter M, Staib G, Kaufmann R
Department of Dermatology, University of Frankfurt Medical School,
Germany.
Parakeratosis variegata is a rare disorder with unknown aetiology.
In a few cases it arises from benign skin diseases such as pityriasis
lichenoides et varioliformis acuta (Mucha Habermann disease) or
pityriasis lichenoides chronica. However, transformation into malignant
diseases such as cutaneous T-cell lymphoma has been observed. We report
an 11-year-old girl with a 10-year history of pityriasis lichenoides
chronica now presenting with parakeratosis variegata. Analysis of skin
infiltrating T cells showed clonally rearranged T-cell receptor gamma
chains occurring with a frequency of more than 2%. This finding is
compatible with the clinical observation of parakeratosis variegata
transforming into a malignant T-cell disorder. We therefore suggest that
patients suffering from parakeratosis variegata and other diseases such
as pityriasis lichenoides et varioliformis acuta or pityriasis
lichenoides chronica should be continuously monitored.
=============================================================
50.) Mucha-Habermann disease and its febrile ulceronecrotic
variant.
=============================================================
Cutis 1996 Aug;58(2):123-31 Related Articles, Books, LinkOut
Tsuji T, Kasamatsu M, Yokota M, Morita A, Schwartz RA
Department of Dermatology, Nagoya City University Medical School,
Nagoya, Japan.
In 1916 Mucha and in 1925 Habermann reported an acute form of
pityriasis lichenoides characterized by the abrupt onset of
papulovesicular eruptions and gave the name, pityriasis lichenoides et
varioliformis acuta (PLEVA) or Mucha-Habermann disease (MH). In 1966,
Degos reported a rare febrile ulceronecrotic variant of MH. MH occurs
mainly in young adults, while febrile ulceronecrotic Mucha-Habermann's
disease (FUMHD) occurs more frequently in children. The etiology of MH
remains obscure, but it may be the result of a hypersensitivity reaction
to an infectious agent. Although clinical and histologic features of the
disease in children are similar to those of adults, more diseases need
to be differentiated in pediatric patients. In addition, a number of
effective therapeutic options in adults with MH are unsuitable for use
in pediatric patients, to whom beginning with oral antibiotics, usually
erythromycin, is recommended. A summary of previously reported fifteen
cases with FUMHD, including our case, is listed.
=============================================================
51.)Febrile ulceronecrotic Mucha-Habermann disease.
=============================================================
J Am Acad Dermatol 1993 Nov;29(5 Pt 2):903-6 Related Articles,
Books, LinkOut
Lopez-Estebaranz JL, Vanaclocha F, Gil R, Garcia B, Iglesias L
Department of Dermatology, 12 de Octubre Hospital, Madrid, Spain.
Febrile ulceronecrotic Mucha-Habermann disease in an 18-year-old
man is reported. This disease is a severe form of pityriasis lichenoides
et varioliformis acuta (PLEVA) and is characterized by the sudden onset
of diffuse coalescent ulcerations associated with high fever and
systemic symptoms. In the present case the disease was preceded by
typical PLEVA. Histologically, a leukocytoclastic vasculitis was seen in
addition to the usual features of PLEVA. Findings of laboratory studies
revealed an elevated erythrocyte sedimentation rate, a high white blood
cell count, and a mild increase in liver enzymes. No systemic
involvement was detected. Findings of T cell receptor gene analysis in
skin and peripheral blood showed no abnormality. The patient was treated
with PUVA and methotrexate with a good response. We review the eight
previously reported cases of febrile ulceronecrotic Mucha-Habermann
disease.
=============================================================
52.) Mucha-Habermann disease: a diagnostic possibility for
prolonged fever associated with systemic and skin symptoms.
=============================================================
Acta Paediatr 1993 Jun-Jul;82(6-7):627-9 Related Articles, Books,
LinkOut
Korppi M, Tenhola S, Hollmen A
Department of Paediatrics, Kuopio University Hospital, Finland.
The severe form of Mucha-Habermann disease with systemic symptoms
is a rarely diagnosed disease which should be considered for children
with prolonged fever, impaired general condition, skin manifestations
and elevated C-reactive protein concentration and/or erythrocyte
sedimentation rate. Eleven cases have been described previously in
children. We describe two acute episodes of this syndrome in a
three-year-old child; the diagnosis was based on clinical,
dermatological and histological findings. During both episodes, the
fever lasted for more than one week, C-reactive protein concentration
increased to more than 150 mg/l, and there was extensive lymph node
enlargement. Skin eruption was initially maculopapulous, then vesiculous
and finally pustulous. On skin biopsy, vasculitic changes were observed.
We treated the second attack of our patient with high-dose gamma
globulin; the first attack appeared to resolve itself spontaneously.
=============================================================
53.) [Acute parapsoriasis in a 5-year-old girl].
=============================================================
Wiad Lek 1990 Apr 1;43(7):308-11 Related Articles, Books,
LinkOut
[Article in Polish]
Kopysc Z, Strehl M
Oddzialu Dzieciecego Wojewodzkiego Szpitala Zespolonego w Zielonej
Gorze.
A case is reported of rarely observed skin changes in a girl aged
5 years. The changes resembled those observed in acute parapsoriasis (p.
lichenoides et varioliformis of Mucha-Habermann). The diagnosis was
established after finding characteristic polymorphic lesions in the form
of papulae, necrotizing vesicles, ulcerations, desquamation of certain
papulae typical of p. guttata, long-term persistence of the lesions and
good general condition of the child. The lesions were situated on the
trunk, and in a lower degree on the face and extremities. Before the
disease the girl hand contact with insecticides (Ovadofox) and
detergents.
=============================================================
54.) Mucha-Habermann's disease and arthritis: possible association
with reactivated Epstein-Barr virus infection.
=============================================================
J Rheumatol 1989 Mar;16(3):387-9 Related Articles, Books,
LinkOut
Edwards BL, Bonagura VR, Valacer DJ, Ilowite NT
Department of Pediatrics, Schneider Children's Hospital of Long
Island Jewish Medical Center, Hyde Park, NY 11042.
We present a 12-year-old girl with skin lesions, arthritis and
clinical response to tetracycline consistent with Mucha-Habermann's
disease. She also showed serological evidence of reactivated
Epstein-Barr virus (EBV) infection. We believe this represents the first
such association of Mucha-Habermann's disease with EBV infection.
=============================================================
55.) [Mucha-Habermann disease. Description of a case in
childhood].
=============================================================
Pediatr Med Chir 1987 May-Jun;9(3):343-5 Related Articles, Books,
LinkOut
[Article in Italian]
Falcini F, Bartolozzi G, Montanelli F, Pratesi G, Taccetti G, Tafi
L, Volpi M, Lotti T
Dipartimento di Pediatria, Universita degli Studi di Firenze,
Italia.
The authors report a case of Mucha-Habermann disease in childhood.
Mucha-Habermann disease is not a very well known, though not infrequent,
disease. It is characterized by recurrent erythematous-papular-vesicular
skin lesions associated with arthralgia or arthritis or large joints.
Prognosis is generally favourable although an evolution towards
Pityriasis Lichenoides Chronica and/or Mycosis Fungoides is possible.
There are not specific laboratory findings for this form. Diagnosis is
essentially based on histology showing an immunopathogenetic vasculitis.
At the present time there is not a safe therapy for the disease; there
are however indications for the use of Erythromycin and we followed
these in our therapy with positive results.
=============================================================
56.) Mucha-Habermann disease in children -- the association with
rheumatic diseases.
=============================================================
J Rheumatol 1982 Mar-Apr;9(2):319-24 Related Articles, Books,
LinkOut
Ellsworth JE, Cassidy JT, Ragsdale CG, Sullivan DB
Two children are described who developed Mucha-Habermann disease
as infants. One boy had juvenile rheumatoid arthritis that ran a
progressive course over 10 years, although his skin disease responded to
a low dose of corticosteroids. One girl had polyarthritis associated
with onset of her rash but both resolved over several years without
treatment. She has since developed scleroderma followed by a
reappearance of her skin lesions.
=============================================================
57.) Mucha-Habermann's disease in children: treatment with
erythromycin.
=============================================================
Arch Dermatol 1978 Nov;114(11):1679-80 Related Articles, Books,
LinkOut
Shavin JS, Jones TM, Aton JK, Abele DC, Smith JG Jr
Safe therapeutic measures for Mucha-Habermann's disease in
children are lacking. Three patients with the disease were treated with
erythromycin for systemic effect. Although the series is small and
uncontrolled, this approach seemed effective. An anti-inflammatory
mechanism related to inhibition of chemotaxis is speculated.
=============================================================
58.) Histiocytic medullary reticulosis presenting as
Mucha-Habermann disease.
=============================================================
Acta Derm Venereol 1978;58(1):57-64 Related Articles, Books,
LinkOut
Freeman MJ, Taylor JS, Levin HS, Dyment PG, Bergfeld WF
Histiocytic medullary reticulosis (HMR) is a rare, progressive,
fatal reticuleondothelial proliferative disorder. It was diagnosed in a
10-year-old boy who had pityriasis lichenoides et varioliformis acuta of
Mucha-Haberman which was controlled by dapsone for 2 years. One month
after cessation of dapsone therapy, cutaneous tumors associated with
fever, lymphadenopathy, and hepatosplenomegaly developed. Tissue biopsy
specimens of skin, liver, spleen, lymph nodes, and a bone marrow
aspirate demonstrated histiocytic erythrophagocytosis and atypical
histiocytosis compatible with HMR. A rapidly progressing, fatal course
followed despite intensive chemotherapy.
=============================================================
59.) Methotrexate for the treatment of Mucha-Habermann disease.
=============================================================
Arch Dermatol 1972 Oct;106(4):507-8 Related Articles, Books,
LinkOut
Cornelison RL Jr, Knox JM, Everett MA
=============================================================
=============================================================
60.)Pityriasis lichenoides-like mycosis fungoides in children.
=============================================================
Br J Dermatol 2000 Feb;142(2):347-52 Related Articles, Books,
LinkOut
Ko JW, Seong JY, Suh KS, Kim ST
Department of Dermatology, Kosin Medical Center, Pusan, South
Korea.
We report three children with clinical features of pityriasis
lichenoides (scaly red to brown papules and macules) in whom there were
histopathological findings of mycosis fungoides (disproportionate
epidermotropism, Pautrier's microabscesses, and wiry and coarse collagen
bundles). Immunohistochemical staining revealed a prevalence of T
lymphocytes in the infiltrate. T-cell receptor gene rearrangement
analysis in lesional skin demonstrated rearrangement of the gamma chain
in all cases. Human T-cell lymphotropic virus type 1 serology was
negative in the two patients in whom this test was performed. Thus,
lesions resembling pityriasis lichenoides can be an unusual and
potentially misleading presentation of mycosis fungoides.
=============================================================
61.) Pityriasis lichenoides in children: clinicopathologic review
of 22 patients.
=============================================================
Pediatr Dermatol 1998 Jan-Feb;15(1):1-6 Related Articles, Books,
LinkOut
Romani J, Puig L, Fernandez-Figueras MT, de Moragas JM
Department of Dermatology, Hospital de la Santa Creu i Sant Pau,
Barcelona, Spain.
Pityriasis lichenoides (PL) is a cutaneous disease of unknown
origin, with an autoinvolutive course, that can occur in pediatric
patients. Traditionally, acute and chronic variants have been described,
but other special forms of presentation have been reported. We reviewed
the clinical records and histopathologic specimens of all pediatric
patients diagnosed with PL in our hospital from 1980 to 1995 to assess
the clinicopathologic features of this disorder in our environment.
Twenty-two of the 118 cases reviewed were pediatric patients less than
15 years old (12 males and 10 females, 18.6% of all patients). Their
ages ranged from 3 to 15 years, with a mean of 9.3 years. Most of the
patients (72%) had the chronic variant of the disease, while the
remainder had an acute course. One patient suffered from acute
ulceronecrotic PL. Systemic treatments prescribed were erythromycin in
eight patients, PUVA in five patients, and methotrexate in one patient.
Three patients had a prolonged course with more than two episodes. Acute
and chronic PL are polar extremes, but individual cases cannot be
classified only on the basis of histopathologic data, since coexistence
of lesions in different stages of evolution can lead to sampling bias.
Acute ulceronecrotic forms and the presence of a variable degree of
cellular atypia in the infiltrate are liable to cause differential
diagnostic problems with lymphomatoid papulosis (LP), which cannot be
completely resolved on the basis of T-cell receptor clonal rearrangement
detection.
=============================================================
62.) The relation between toxoplasmosis and pityriasis lichenoides
chronica.
=============================================================
J Egypt Soc Parasitol 1997 Apr;27(1):93-9 Related Articles, Books,
LinkOut
Nassef NE, Hammam MA
Department of Parasitology, Faculty of Medicine, Menoufia
University, Egypt.
Pityriasis lichenoides chronica (PLC) is a rare skin disease of
uncertain aetiology. Many infectious agents have been incriminated as
the cause of the disease. One of these agents is toxoplasmosis. The aim
of this work was to find out if there is a relationship between
toxoplasmosis and PLC. Twenty two patients (17 males and 5 females)
diagnosed clinically and histopathologically as PLC were chosen for this
study. Also twenty apparently healthy individuals free from skin lesions
were included as a control group. Patients and controls were examined
clinically for signs of toxoplasmosis and submitted for indirect
haemagglutination (IHA) and indirect immunofluorescent antibody (IFA)
tests in our Parasitology laboratory for serodiagnosis of toxoplasmosis.
Toxoplasmosis was diagnosed in 8 (36.36%) and 3 (15%) in PLC patients
and controls respectively by both tests. Using pyrimethamine and
trisulfapyrimidine in treating PLC patients, showed subsidence of skin
lesions in five patients with toxoplasmosis within two months from the
beginning of therapy. The remaining patients showed no response to
treatment. On conclusion, toxoplasmosis appears to play a role in the
aetiology of PLC and serological tests for diagnosing toxoplasmosis
should be performed in all PLC patients.
=============================================================
63.) Experience with UVB phototherapy in children.
=============================================================
Pediatr Dermatol 1996 Sep-Oct;13(5):406-9 Related Articles, Books,
LinkOut
Tay YK, Morelli JG, Weston WL
Department of Pediatric Dermatology, University of Colorado Health
Sciences Center, Denver 80262, USA.
Twenty children age 14 months to 12 years with photoresponsive
dermatoses were treated with ultraviolet B (UVB) phototherapy over four
years. Ten children had psoriasis, five had pityriasis lichenoids, and
five had atopic dermatitis. All received short courses (average 34
treatments) of phototherapy with either no maintenance or short
maintenance. Treatment was effective and well tolerated in most
patients, and no serious side effects were seen. Patients with psoriasis
and pityriasis lichenoides cleared completely. No patient with atopic
dermatitis cleared completely, but all were moderately improved, with
reduction of the extent of eczema and decreased pruritus. It appears
that UVB phototherapy is a valuable and safe therapeutic option for
selected children who do not respond to other treatments.
=============================================================
64.) Pityriasis lichenoides of childhood with atypical
CD30-positive cells and clonal T-cell receptor gene rearrangements.
=============================================================
J Am Acad Dermatol 1996 Sep;35(3 Pt 1):489-90 Related Articles,
Books, LinkOut
Panhans A, Bodemer C, Macinthyre E, Fraitag S, Paul C, de Prost Y
Dermatology, Hematology, Hopital Necker, Paris, France.
Comments:
Comment in: J Am Acad Dermatol 1997 Aug;37(2 Pt 1):287
=============================================================
============================================================
65.) Pityriasis lichenoides et varioliformis acuta and group-A
beta hemolytic streptococcal infection.
============================================================
AU: English-JC-3rd; Collins-M; Bryant-Bruce-C
AD: Department of Primary Care and Community Medicine, USA MEDDAC,
Ft.
Campbell, Kentucky 42223-5349, USA.
SO: Int-J-Dermatol. 1995 Sep; 34(9): 642-4
Letter
=====================================================
66.) Treatment of adult diffuse pityriasis lichenoides chronica
with narrowband ultraviolet B: experience and literature review.
=====================================================
Clin Exp Dermatol. 2017 Jan 23. doi: 10.1111/ced.13035. [Epub
ahead of print]
Fernández-Guarino M1, Aboin-Gonzalez S1, Ciudad Blanco C1,
Velázquez Tarjuelo D1, Lázaro Ochayta P1.
Author information
1Dermatology Department, Hospital Universitario Sanitas La
Zarzuela, Universidad Francisco de Vitoria, Madrid, Spain.
Abstract
Pityriasis lichenoides chronica (PLC) is an infrequent dermatosis
of unknown aetiology, wholse evolution and response to treatment differs
between children and adults. When PLC is recalcitrant or unresponsive to
topical treatment, phototherapy is one of the main treatments used. We
carried out a prospective study of adult diffuse PLC treated with
narrowband ultraviolet B (NB-UVB). We treated eight patients whose
disease showed no response to topical therapy, and obtained a complete
response rate of 88% in a mean of 23 sessions (cumulative dose 16.99
J/cm2 ). However, the relapse rate was 43% in the first 6 months. Our
results are similar to those of other published studies but there is
much variability between them in the doses applied and the number of
sessions needed. Further studies are necessary to devise a protocol for
NB-UVB treatment of PLC.
=================================================
67.) Phototherapy in children: Considerations and indications.
================================================
Clin Dermatol. 2016 Sep-Oct;34(5):633-9. doi:
10.1016/j.clindermatol.2016.05.018. Epub 2016 May 24.
Crall CS1, Rork JF2, Delano S1, Huang JT3.
Author information
1Harvard Medical School, Dermatology Program, Division of Allergy
and Immunology, Department of Medicine, Boston Children's Hospital,
Boston, MA.
2Department of Dermatology, University of Massachusetts School of
Medicine, Worcester, MA.
3Harvard Medical School, Dermatology Program, Division of Allergy
and Immunology, Department of Medicine, Boston Children's Hospital,
Boston, MA. Electronic address: Jennifer.huang@childrens.harvard.edu.
Abstract
Phototherapy can be a safe and effective treatment for various
skin diseases in children. Special considerations governing the use of
this treatment modality in pediatric populations include patient,
family, and facility-based factors that are oriented around heightened
concerns with regard to safety and tolerability of treatment. Although
phototherapy has been found to be effective in a wide range of
dermatologic conditions affecting pediatric populations, including
psoriasis, atopic dermatitis, pityriasis lichenoides, cutaneous T-cell
lymphoma, and vitiligo, there is need for additional research on other
conditions in which phototherapy has shown promise.
================================================
68.) Phototherapy for Pityriasis Lichenoides in the Pediatric
Population: A Review of the Published Literature.
===============================================
Am J Clin Dermatol. 2016 Dec;17(6):583-591
Maranda EL1, Smith M2, Nguyen AH2, Patel VN3, Schachner LA3,
Joaquin JJ3.
Author information
1Department of Dermatology and Cutaneous Surgery, University of
Miami Miller School of Medicine, 1475 NW 12th Ave., Miami, FL, 33136,
USA. emaranda@med.miami.edu.
2Creighton University School of Medicine, Omaha, NE, USA.
3Department of Dermatology and Cutaneous Surgery, University of
Miami Miller School of Medicine, 1475 NW 12th Ave., Miami, FL, 33136,
USA.
Abstract
BACKGROUND:
Pityriasis lichenoides (PL) is a dermatologic disorder that
manifests in either the acute (pityriasis lichenoides et varioliformis
acuta) or the chronic form (pityriasis lichenoides chronica, also known
as parapsoriasis chronica). Traditional first-line therapy consists of
corticosteroids or antibiotics; however, these treatments are often
accompanied with multiple side effects and may be ineffective.
OBJECTIVE:
The goal of this study was to review the use of phototherapy for
treating PL in the pediatric population.
MATERIALS AND METHODS:
We performed a systematic review of the literature in the National
Library of Medicine's PubMed database and the SCOPUS database discussing
phototherapy for treatment of PL in the pediatric population. The
following search terms were used: 'pityriasis lichenoides', 'pityriasis
lichenoides chronica', 'pityriasis lichenoides et varioliformis acuta',
and 'febrile ulceronecrotic Mucha-Habermann disease'.
RESULTS:
The systematic search and screening of articles resulted in 14
articles including a total of 64 patients with PL treated with
phototherapy. Three different modalities were utilized, with five
studies using broadband ultraviolet B (BB-UVB) radiation, nine studies
utilizing narrowband UVB (NB-UVB), and two studies employing psoralen
with ultraviolet A (PUVA) therapy. Overall, the use of BB-UVB had an
initial clearance rate of 89.6 % with 23.1 % recurrence, whereas NB-UVB
cleared 73 % of the lesions with no recurrence, and PUVA therapy
initially cleared 83 % of the lesions with 60 % recurrence. The
side-effect profiles were similar and revealed limited toxicity.
CONCLUSION:
Phototherapy shows promising results and a favorable side-effect
profile in the treatment of PL. Ultimately, large randomized controlled
trials are needed to determine optimal treatments.
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69.) [Febrile ulceronecrotic Mucha-Habermann disease].
=================================================
Rev Med Chil. 2016 Sep;144(9):1214-1217. doi:
10.4067/S0034-98872016000900017.
[Article in Spanish]
Arellano Lorca J1, Yáñez Silva I2, Soto Vilches F3, Luna Heine A1,
Corredoira Salum Y4.
Author information
1Servicio de Dermatología, Hospital Clínico San Borja Arriarán,
Santiago, Chile.
2Universidad Católica del Maule, Chile.
3Departamento de Dermatología, Universidad de Chile, Santiago,
Chile.
4Anatomía Patológica, Hospital Clínico San Borja Arriarán,
Universidad de Chile, Santiago, Chile.
Abstract
Pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis
lichenoides chronica (PLC) and febrile ulceronecrotic Mucha-Habermann
disease (FUMHD) are considered different manifestations of the same
disease. Febrile ulceronecrotic Mucha-Habermann disease is a rare, and
potentially lethal illness which is characterized by fast progression of
numerous papules that converge, ulcerate and form a plaque with a
necrotic center, together with hemorrhagic vesicles and pustules that
are associated with high fever and variable systemic symptoms. We report
a 16 years old male presenting with erythematous papules with crusts and
fever. The diagnosis of febrile ulceronecrotic Mucha-Habermann disease
was confirmed with the pathological study of the lesions. He was
successfully treated with minocycline after a failed attempt of
treatment with prednisone.
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70.) Pityriasis Lichenoides in Childhood: Review of Clinical
Presentation and Treatment Options.
================================================
Pediatr Dermatol. 2015 Sep-Oct;32(5):579-92. doi:
10.1111/pde.12581. Epub 2015 Mar 26.
Geller L1,2, Antonov NK3, Lauren CT4,5, Morel KD4,5, Garzon MC4,5.
Author information
1Department of Dermatology, Icahn School of Medicine at Mount
Sinai, New York, New York.
2Department of Pediatrics, Icahn School of Medicine at Mount
Sinai, New York, New York.
3College of Physicians and Surgeons, Columbia University, New
York, New York.
4Department of Dermatology, Columbia University, New York, New
York.
5Department of Pediatrics, Columbia University, New York, New
York.
Abstract
Pityriasis lichenoides (PL) is a skin condition of unclear
etiology that occurs not uncommonly in childhood. It is often classified
into the acute form, pityriasis lichenoides et varioliformis acuta
(PLEVA), and the chronic form, pityriasis lichenoides chronica (PLC). We
performed a comprehensive review of the English-language literature
using the PubMed database of all cases of childhood PL reported from
1962 to 2014 and summarized the epidemiology, clinical features,
treatment options, and prognosis of this condition in children. The
proposed etiologies are discussed, including its association with
infectious agents, medications, and immunizations and evidence for PL as
a lymphoproliferative disorder. We found an average age of PL onset of
6.5 years, with a slight (61%) male predominance. We also found that
PLEVA and PLC tend to occur with equal frequency and that, in many
cases, there is clinical and histopathologic overlap between the two
phenotypes. When systemic therapy is indicated, we propose that oral
erythromycin and narrowband ultraviolet B phototherapy should be
first-line treatment options for children with PL since they have been
shown to be effective and well tolerated. In most cases, PL follows a
benign course with no greater risk of cutaneous T-cell lymphoma,
although given the rare case reports of transformation, long-term
follow-up of these patients is recommended.
Produced by Dr. Jose Lapenta R. Dermatologist
Maracay Estado Aragua Venezuela
2.017 -2025
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