The Pityriasis Lichenoides, a paraneoplastic syndrome?
The Pityriasis Lichenoides, a paraneoplastic syndrome?
La Pitiriasis liquenoide, un síndrome paraneoplasico?
EDITORIAL ENGLISH
=================
Hello friends of the net, in this DERMAGIC EXPRESS edition the topic is quite
interesting, the LICHENOID PITYRIASIS,(PL) and its VARIANTS, really a
paraneoplastic syndrome ???.
In the year of 1.916 MUCHA described the ACUTE form of this disease for the
first time, in 1925 HABERMANN he also made the same thing, being known since
then with the name of PITYRIASIS-LICHENOIDES-ET-VARIOLIFORMIS-ACUTE- (MUCHA
HABERMANN'S DISEASE-PLEVA). In the year of 1.996 DEGOS described a febrile
ULCERONECROTIC form (FUMH) of the classic illness of MUCHA-HABERMAN.
There is also described a second variant: the CHRONIC form OF of the
disease. The CHRONIC PITYRIASIS LICHENOIDES (PLC).
Disease of difficult treatment and uncertain behavior, of unknown cause,
associated in some occasions as a previous state to
malignancy (lymphoma), also
being associated to the
linfomatoid papulosis
which has also been related with
cutaneous lymphomas (CTCL)
In this bibliographical review I found some interesting aspects that I
highlight next:
1.) Exist 2 types of the disease:
A.) PITYRIASIS-LICHENOIDES-ET-VARIOLIFORMIS-ACUTE
(Mucha-Habermann) 1.916-
1925- PLEVA).
The Acute form has a variant:
The febrile ulceronetrotic type of Mucha-Habermann (1.966 (Degos)-(FUMH)
2.) Both variants have been associated to malignancy (lymphoma).
3.) They can be presented both in children and
adults.
4.) A bigger association has been observed with malignancy in the adults
than in children.
5.) Cases of remission have been described after tonsillectomy.
6.) Cases have been described in oneself family.
7.) Three cases were described in children where histopathological changes
compatible with mycosis fungoides were found in biopsies.
8.) Also has been described cases of chronic pityriasis lichenoide that
becomes in paraqueratosis variegata. (parapsoriasis).
9.) The ACUTE form can disappear or to evolve toward the Chronic form or
mycosis fungoides.
10.) The chronic form can appear for the first time as chronic variant
(PLC), wich disappear or evolve to
linfomatoid papulosis - lymphoma.
11.) Among the possible causes a possible hypersensitivity reaction is
mentioned to an infectious agent, among them Epstein Barr's virus,
Toxoplasma Gondii. and streptococcal infection.
12.) In a study made in egypt in 1.997 on 22 patients with PLC, IT WAS
FOUND that 36.36% had toxoplasmosis.
13.) It has also been described in association to rheumatic DISEASE:
reumatoid arthritis.
14.) It has found deposits of IGM and C3 in biopsies of patient with
LICHENOID PITYRIASIS ACUTE AND CHRONIC. A DISEASE BY immune COMPLEX
?
15.) There is not definitive treatment, but tetracycline, erythromycin,
methotrexate, Pentoxifylline and ultraviolet light ARE THE most frequently USED.
Based on these interesting data and EVIDENTS we could consider
pityriasis lichenoid
and its variants as a SYNDROME very close to the PARANEOPLASIA which we must
always watch for its possible evolution to
malignancy.
Between routine examinations, always ask for titles of Toxoplasma, Epstein
Barr virus and immunological tests: C3-C4 CH 50, Immunoglobulins (IGM),
antistreptolysin-O (ASO), etc.
In these 70 references you will know the disease and its variants, association
with malignancy and its therapeutic alternatives.
Greetings to all.
Dr. José Lapenta.
EDITORIAL ESPAÑOL
==================
Hola amigos de la red, en esta edición del DERMAGIC/EXPRESS el tema es
bastante interesante, la PITIRIASIS LIQUENOIDE y sus VARIANTES, realmente un
síndrome para neoplásico ???.
En el año de 1.916 MUCHA describió por primera vez la forma AGUDA de esta
enfermedad, en 1925 HABERMANN también hizo lo mismo, siendo desde entonces
conocida con el nombre de PITIRIASIS LIQUENOIDE VARIOLIFORME AGUDA DE MUCHA
HABERMANN.(PLEVA) En el año de 1.996 DEGOS describio una forma febril y
ULCERONECROTICA de la clasica enfermedad de MUCHA HABERMAN.(FUMH)
Tambien hay descrita una segunda variante: la forma CRONICA DE de la
enfermedad.
La pitiriasis liquenoide cronica (PLC).
Enfermedad de dificil tratamiento y comportamiento incierto, de causa
desconocida, se ha asociado en algunas ocasiones como un estado previo a
malignidad (linfoma), tambien siendo asociada a la
papulosis linfomatoide la cual a su vez tambien ha sido relacionada con
linfomas cutaneos
(CTCL)
En esta revision bibliografica encontre algunos aspectos interesantes que
resalto a continuacion:
1.) Existen 2 tipos de la enfermedad:
1.) Existen 2 tipos de la enfermedad:
A.) Pitiriasis Liquenoide varioliforme Aguda (Mucha Habermann 1.916-
1925-PLEVA).
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 asociacion con malignidad en los adultos que
en niños.
5.) Se han descrito casos de remision despues de tonsilectomia.
6.) Se han descrito casos en una misma familia.
7.) Se describieron tres casos en niños donde se encontro en las biopsias
cambios histopatologicos compatibles con micosis fungoides.
8.)Tambien se han descrito casos de pitiriasis liquenoide cronica que se
transformo en paraqueratosis variegata. (parapsoriasis).
9.) La forma aguda puede desaparecer o evolucionar hacia la forma cronica o
micosis fungoides.
10.) la forma cronica puede aparecer como cronica por primera vez,
desaparecer o evolucionar a papulosis linfomatoide- linfoma.
11.) Entre las posibles causas etiologicas se menciona un posible estado de
hiperreactividad a un agente infeccioso, entre ellos el virus de Epstein
Barr, Toxoplasma Gondii e infeccion estreptococcica.
12.) En un estudio hecho en egipto en 1.997 sobre 22 pacientes con PLC se
consiguio que el 36.36% tenia toxoplasmosis.
13.) Tambien ha sido descrita en asociacion a enfermedades reumaticas:
artritis reumatoide.
14.) Se han encontrado depositos de IGM y C3 en biopsias de pacientes con
PITIRIASIS LIQUENOIDE AGUDA Y CRONICA. Una enfermedad por complejos inmunes
?
15.) No hay tratamiento especifico pero las tetraciclinas, eritromicina
metotrexato, pentoxifilina y luz ultravioleta son las mas
usados.
En base a estos datos interesantes y EVIDENTES podriamos considerar a la
pitiriasis liquenoide y sus variantes como un SINDROME muy cercano a la PARANEOPLASIA el cual
debemos vigilar siempre por su posible evolucion a
malignidad.
Entre los examenes de rutina, pedir siempre titulos de Toxoplasma,
Epstein Barr virus y examenes inmunologicos: C3, C4 CH 50 Inmunoglobulinas
(IGM), antistreptolisina -O (ASTO), etc.
En estas 70 referencias conocerás la enfermedad y sus variantes, la asociación
con malignidad y sus alternativas terapéuticas.
Saludos a Todos. !!!
Dr. Jose Lapenta.
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REFERENCIAS BIBLIOGRAFICAS /
BIBLIOGRAPHICAL REFERENCES
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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.
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12.) Lymphomatoid papulosis: clinicopathological comparative study
with pityriasis lichenoides et varioliformis acuta.
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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.
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13.)Comparative clinicopathological study on pityriasis lichenoides
chronica and small plaque parapsoriasis.
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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.
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14.) Severe febrile Mucha-Habermann's disease in children: case report
and review of the literature.
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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.
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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.
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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.
==================================================
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.
================================================
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
Telf: 02432327287-02432328571
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