La Pitiriasis liquenoide, un sindrome paraneoplasico ???

DATA-MEDICOS 
DERMAGIC/EXPRESS No 2-96
03 Diciembre 2000, 03 December 2.000

~ La pitiriasis Liquenoide y sus variantes un sindrome paranaoplasico ???~ 
~ The Pityriasis Lichenoides and its variants, a paraneoplastic syndrome ???~ 

EDITORIAL ESPANOL
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Hola amigos de la red, en esta primera edicion del DERMAGIC/EXPRESS en el WEB site el tema es bastante interesante, la PITIRIASIS LIQUENOIDE y sus VARIANTES, realmente un sindrome paraneoplasico ???. 

En el a�o de 1.916 MUCHA describio por primera vez la forma AGUDA de esta enfermedad, en 1925 HABERMANN tambien hizo lo mismo, siendo desde entonces conocida con el nombre de PITIRIASIS LIQUENOIDE VARIOLIFORME AGUDA DE MUCHA HABERMANN.(PLEVA) En el a�o 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: 

A.) Pitiriasis Liquenoide varioliforme Aguda (Mucha Habermann 1.916- 1925-PLEVA). La Aguda tiene una variante: La Forma febril y ulceronetrotica de Mucha Habermann (1.966 (Degos) (FUMH) 

B.) Pitiriasis liquenoide Cronica. (PLC)

2.) Ambas variantes han sido asociada a estados malignos (linfoma) 
3.) Se pueden presentar tanto en ni�os como adultos. 
4.) Se ha observado una mayor asociacion con malignidad en los adultos que en ni�os. 
5.) Se han descrito casos de remision despues de tonsilectomia. 
6.) Se han descrito casos en una misma familia. 
7.) Recientemente se describieron en febrero del 2.000 tres casos en ni�os 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 y entre los examenes de rutina, pedir siempre titulos de Toxoplasma,  Epstein Barr virus y examenes inmunologicos: C3-Inmunoglobulinas (IGM),etc 

En las 65 referencias los hechos.... 

Saludos a Todos.  !!! Dr. Jose Lapenta R.

EDITORIAL ENGLISH
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Hello friends of the net, in this first edition of the DERMAGIC/EXPRESS in the WEB site 
is the topic quite interesting, the LICHENOID PITYRIASIS, and its VARIANTS, really a paraneoplastic syndrome ???. 

In the year of 1.916 MUCHA described the ACUTE form of this illness for the first time, n 1925 HABERMANN he also made the same thing, being known from then on with the name of PITYRIASIS-LICHENOIDES-ET-VARIOLIFORMIS-ACUTA- (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 illness. The CHRONIC PITYRIASIS LICHENOIDES (PLC). 

Illness of difficult treatment and uncertain behavior, of unknown cause, she has associated in some occasions as a previous state to malignancy (lymphoma), also being associated to the linfomatoid papulosis the one which in turn has also been related with cutaneous lymphomas (CTCL)

In this bibliographical revision I found some interesting aspects that I stand out next: 
1.) 2 types of the illness exist: 
A.) Pitiriasis Liquenoide 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) 

B.) Chronic Pityriasis lichenoides.(PLC) 

2.) Both variants have been associated to malignancy (lymphoma) 
3.) They can be presented so much in children as adults. 
4.) A bigger association has been observed with malignancy in the adults that in children. 
5.) Cases of remission have been described after tonsillectomy. 
6.) Cases have been described in oneself family. 
7.) Recently they were described in February of the 2.000 three cases in children where it was in the biopsies histopathological changes of mycosis fungoides. 
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, to disappear or to 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 was gotten that 36.36% had toxoplasmosis. 
13.) It has also been described in association to rheumatic illnesses: arthritis reumatoid. 
14.)It has found deposits of IGM and C3 in biopsies of patient with LICHENOID PITYRIASIS ACUTE AND CHRONIC. An illness for complex immune? 
15.) There is not definitive treatment, but tetracycline, erythromycin, methotrexate, and ultraviolet light plows used most frequently 

Based on these interesting an EVIDENT data we could consider to the LICHENOIDES PITYRIASIS and their variants as a very near to an PARANEOPLASTIC SYNDROME wichh we should always watch over for their possible evolution to malignancy and among the routine exams, to always request: toxoplasma, Epstein Barr virus, and Immunologic exams: C3, IGM, etc. 

In the 65 references the facts.... 

Greetings to all !!! Dr Jose Lapenta.

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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. 
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1.) Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis =============================================================
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 =============================================================
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.

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16.) UV-B phototherapy for pityriasis lichenoides.
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SO - Australas J Dermatol 1985 Apr;26(1):9-13
AU - Siew NT
PT - JOURNAL ARTICLE
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17.) [Parakertosis variegata after pityriasis lichenoides et varioliformis acuta]
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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.

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18.) Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta.
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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
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19.) Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta.
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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.

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20.) [Pityriasis-lichenoides-et-varioliformis-acuta-like drug exanthema caused by astemizole]
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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.

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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.

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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.

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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.

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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.

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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.

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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 

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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
=============================================================

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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48.) Lichenoid pityriasis. Clinical study of 13 cases].
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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.

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49.) The transformation of pityriasis lichenoides chronica into parakeratosis variegata in an 11-year-old girl.
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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.

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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.

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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.

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52.) Mucha-Habermann disease: a diagnostic possibility for prolonged fever associated with systemic and skin symptoms.
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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.

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53.) [Acute parapsoriasis in a 5-year-old girl].
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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.

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54.) Mucha-Habermann's disease and arthritis: possible association with reactivated Epstein-Barr virus infection.
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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.

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55.) [Mucha-Habermann disease. Description of a case in childhood].
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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.

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56.) Mucha-Habermann disease in children -- the association with rheumatic diseases.
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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.

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57.) Mucha-Habermann's disease in children: treatment with erythromycin.
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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.

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58.) Histiocytic medullary reticulosis presenting as Mucha-Habermann disease.
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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.

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59.) Methotrexate for the treatment of Mucha-Habermann disease.
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Arch Dermatol 1972 Oct;106(4):507-8 Related Articles, Books, LinkOut 

Cornelison RL Jr, Knox JM, Everett MA
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60.)Pityriasis lichenoides-like mycosis fungoides in children.
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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.

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61.) Pityriasis lichenoides in children: clinicopathologic review of 22 patients.
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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.

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62.) The relation between toxoplasmosis and pityriasis lichenoides chronica.
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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.

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63.) Experience with UVB phototherapy in children.
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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.

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64.) Pityriasis lichenoides of childhood with atypical CD30-positive cells and clonal T-cell receptor gene rearrangements.
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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 
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65.) Pityriasis lichenoides et varioliformis acuta and group-A beta hemolytic streptococcal infection. 
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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
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                           ACUTE PITYRIASIS LICHENOIDES (MUCHA HABERMANN)