Pitiriasis liquenoide varioliforme aguda posterior al inicio de eritropoyetina / Acute varioliform lichenoid pityriasis following the initiation of erythropoietin

Resumen La pitiriasis liquenoide varioliforme aguda (PLEVA) pertenece a un grupo de enfermedades categorizadas como pitiriasis liquenoides, junto con la enfermedad febril úlcero-necrótica de Mucha-Habermann y la pitiriasis liquenoide crónica (PLC). Se caracteriza por la aparición aguda de múltiples pápulas eritemato-violáceas con posterior necrosis, discromía residual y cicatrices varioliformes. Dentro de las teorías patogénicas propuestas se encuentra el posible papel de agentes infecciosos, trastornos linfoproliferativos, complejos inmunes e incluso, asociación a medicamentos. Se presenta un casode una mujer adulta con un cuadro típico de PLEVA con confirmación histopatológica, cuyas lesiones aparecieron posteriormente al inicio de eritropoyetina.

Abstract Pityriasis lichenoides et varioliformes acuta (PLEVA) is part of a group of diseases clustered as pityriasis lichenoides, next to febrile ulceronecrotic Mucha-Habermann disease and pityriasis lichenoides chronica. It's characterized by a sudden onset of multiple erythematous and violaceous papules which develop necrosis, leaving residual dyschromia and varioliform scars. It's been hypothesized the possible role of infectious agents, lymphoproliferative diseases, immune complexes and drugs. We present the case of a woman with a typical PLEVA with histopathological confirmation, whose lesions appeared after therapy with erythropoietin.

Pitiríase liquenoide pós-vacinação contra COVID-19: um relato de caso / Pityriasis lichenoides after COVID-19 vaccination: a case report

O artigo aborda o primeiro relato de caso que associa o desenvolvimento de pitiríase liquenoide com a vacinação contra a COVID-19. Em uma revisão literária foram encontrados escassos estudos que associam a pitiríase liquenoide como reação a outras vacinas. O mecanismo de desenvolvimento da doença ainda não é bem conhecido. Sabe-se apenas que se trata de uma reação inflamatória imunomediada. O diagnóstico da pitiríase liquenoide é clínico e é considerado um desafio, devido ao grande número de diagnósticos diferenciais e das diferentes formas de apresentação da doença. Desse modo, a maioria dos casos exige amparo na biópsia e em exames laboratoriais. As opções terapêuticas podem incluir o uso de antibióticos e imunossupressores. Destaca-se ainda a efetividade da fototerapia como tratamento de escolha da pitiríase liquenoide, podendo proporcionar uma resolução quase que completa das lesões e não causar efeitos sistêmicos que outras terapias poderiam trazer.

This study addresses the first case report of pityriasis lichenoides development after COVID-19 vaccination. A literature review found few studies describing pityriasis lichenoides as an adverse reaction to other vaccines. Although it is an immune-mediated inflammatory response, the development mechanism of this disease remains not well known. The diagnosis of pityriasis lichenoides is clinical and is considered a challenge due to the considerable number of differential diagnoses and the different forms of presentation of the disease. Thus, most cases require confirmation by biopsy and laboratory tests. Therapeutic options may include the use of antibiotics and immunosuppressants. The effectiveness of phototherapy is also highlighted as the treatment of choice for pityriasis lichenoides, as it can promote an almost complete resolution of lesions without causing systemic effects, unlike other therapies.

A Resposta da pitiríase liquenoide varioliforme aguda à fototerapia ­ um relato de caso / The response of acute pityriasis lichenoid varioliformis to phototherapy ­ a case report

A pitiríase liquenóide e varioliforme aguda (PLEVA), é classificada dentro do grupo de dermatoses crônicas, idiopáticas, chamado de "parapsoríases". Caracteriza-se por erupção aguda ou subaguda de múltiplas e pequenas pápulas eritematosas, que podem evoluir com vesiculação e necrose hemorrágica central. A fototerapia possui altos níveis de eficácia e tolerabilidade em uma variedade de doenças inflamatórias e neoplásicas da pele caracterizadas por infiltrados epiteliais e dérmicos rico em linfócitos T . Este artigo relata o caso de uma mulher de meia idade com apresentação clínica típica e diagnóstico histopatológico de PLEVA. Por se tratar de doença que nem sempre responde a tratamentos sistêmicos, o objetivo da proposta terapêutica com fototerapia visou suprimir a doença por seu efeito anti-inflamatório e imunossupressor e prevenir a evolução para o linfoma cutâneo de células T.

Pityriasis lichenoid et varioliformis acute (PLEVA) is classified within the group of chronic, idiopathic dermatoses called "parapsoriasis". It is characterized by an acute or subacute eruption of multiple small erythematous papules, which may progress to vesiculation and central hemorrhagic necrosis. Phototherapy has high levels of efficacy and tolerability in a variety of inflammatory and neoplastic skin diseases characterized by epithelial and dermal infiltrates rich in T lymphocytes. This article reports the case of a middle-aged woman with typical clinical presentation and histopathological diagnosis of PLEVA. As it is a disease that does not always respond to systemic treatments, the objective of the therapeutic proposal with phototherapy was to suppress the disease due to its anti-inflammatory and immunosuppressive effect and prevent the evolution to cutaneous T-cell lymphoma.

A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities

Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose as in the early course they can mimic erythema multiforme or lymphomatoid papulosis. We report a case of a 38-year-old woman who presented with 90% body surface area involvement, fever, diarrhea, malaise and associated comorbidities. She was treated with systemic steroids and methotrexate but suffered a fatal outcome. So far, a total of 65 cases are reported in the literature.

Clinicopathological analysis of 81 cases of parapsoriasis / 中华皮肤科杂志

Objective@#To analyze clinicopathological features of parapsoriasis.@*Methods@#Clinical and pathological data were collected from 81 patients with parapsoriasis in Department of Dermatology, Peking University First Hospital between January 2016 and May 2018, and analyzed retrospectively.@*Results@#Among the 81 patients with parapsoriasis, 44 were male and 37 were female, with age ranging from 6 to 77 years. Their disease course ranged from 7 days to 30 years, and the median disease course was 12 months. Moreover, 61 (75.3%) patients were aged less than 40 years, and 20 (24.7%) were aged 41 years and older. Of the 81 patients, 16 (19.8%) were diagnosed with small plaque parapsoriasis, 20 (24.7%) with large plaque parapsoriasis, 37 (45.7%) with pityriasis lichenoides chronica, and 8 (9.9%) with pityriasis lichenoides et varioliformis acuta. Additionally, distribution patterns of lesions included diffuse type in 65 cases (80.2%) , central type in 6 cases (7.4%) , and peripheral type in 10 cases (12.3%) . Histopathological examination of skin lesions revealed liquefaction degeneration of basal cells in 69 cases (85.2%) , migration of lymphocytes into the epidermis in 67 cases (82.7%) , focal parakeratosis in 42 cases (51.9%) , keratinocyte necrosis in 29 cases (35.8%) , extravasation of erythrocytes in 23 cases (28.4%) , epidermal spongiosis in 21 cases (25.9%) , and dermal perivascular focal infiltration in 61 cases (75.3%) .@*Conclusion@#Parapsoriasis has characteristic clinical and pathological manifestations, and a close combination of clinical manifestations with pathological features is necessary for its accurate diagnosis.

Pitiriasis liquenoide crónica en un adulto mayor: reporte de un caso / Pityriasis lichenoides in an older adult: report of a case

La pitiriasis liquenoide crónica (PLC) es una enfermedad benigna, que se caracteriza por tener un curso gradual, con aparición de múltiples pápulas con escamas adherentes de predominio en tronco y extremidades proximales. Con el objetivo de describir la presentación clínica, discutir el diagnóstico, diagnósticos diferenciales, tratamiento y revisar la literatura se presenta el caso de un hombre de 88 años que presentó múltiples lesiones papuloescamosas en extremidades inferiores. Se realizó biopsia cutánea que confirmó el diagnóstico de PLC. El paciente recibió tratamiento con emulsión hidratante, clobetasol tópico y claritromicina con resolución completa de las lesiones. La pitiriasis liquenoide crónica es un trastorno inflamatorio poco frecuente, de etiología desconocida que ocurre más comúnmente en adultos jóvenes y niños. Si bien la clínica es sugerente, se requiere biopsia cutánea para su confirmación diagnóstica. Es un trastorno benigno, a menudo asintomático y autolimitado, por lo que se debe valorar su tratamiento paciente a paciente. Principalmente se utilizan corticoides tópicos y antibióticos orales (tetraciclinas y eritromicina). Se ha vinculado en escasas publicaciones con el desarrollo de linfoma cutáneo y como síndromes paraneoplásicos, por lo que se sugiere realizar seguimiento.

Chronic lichenoid pityriasis (PLC) is a benign disorder, characterized by a gradual course with the appearance of multiple squamous papules with adherent scales predominating in the trunk and proximal extremities. With the objective to describe its clinical presentation, diagnosis, differential diagnosis, treatment and review literature, we present an 88-yearold male with multiple lesions in the lower extremities of one year of evolution, with papules and adherent scales. A skin biopsy was performed that confirmed the diagnosis of PLC. The patient received treatment with moisturizing emulsion, clobetasol topical and clarithromycin with complete resolution of the lesions. Chronic lichenoid pityriasis is a rare inflammatory disease of unknown etiology that occurs most commonly in young adults and children. Although the clinic is suggestive, a skin biopsy is required for diagnostic confirmation. It is a benign disorder, often asymptomatic and self-limiting, so its patientto- patient treatment should be assessed. Topical corticosteroids and oral antibiotics (tetracyclines and erythromycin) are used. It has been linked in few publications with the development of cutaneous lymphoma and as paraneoplastic syndromes, so it is suggested to follow up.

Enfermedad de Mucha-Habermann febril ulceronecrótica con respuesta favorable a minociclina, a propósito de un caso / Febrile ulceronecrotic Mucha-Habermann disease: report of one case

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.

Pityriasis Lichenoides-like Mycosis Fungoides: Clinical and Histologic Features and Response to Phototherapy

BACKGROUND: Pityriasis lichenoides (PL)-like skin lesions rarely appear as a specific manifestation of mycosis fungoides (MF). OBJECTIVE: We investigated the clinicopathological features, immunophenotypes, and treatments of PL-like MF. METHODS: This study included 15 patients with PL-like lesions selected from a population of 316 patients diagnosed with MF at one institution. RESULTS: The patients were between 4 and 59 years of age. Four patients were older than 20 years of age. All of the patients had early-stage MF. In all patients, the atypical lymphocytic infiltrate had a perivascular distribution with epidermotropism. The CD4/CD8 ratio was <1 in 12 patients. Thirteen patients were treated with either narrowband ultraviolet B (NBUVB) or psoralen+ultraviolet A (PUVA), and all of them had complete responses. CONCLUSION: PL-like MF appears to have a favorable prognosis and occurrence of this variant in adults is uncommon. MF should be suspected in the case of a PL-like skin eruption. Therefore, biopsy is required to confirm the diagnosis of PL-like MF, and NBUVB is a clinically effective treatment.

Pityriasis lichenoides et varioliformis acuta after influenza vaccine

The etiology of pityriasis lichenoides is unknown. One of the accepted theories admits that PL is an inflammatory response to extrinsic antigens such as infectious agents, drugs and vaccines. In recent medical literature, only the MMR vaccine (Measles, Mumps and Rubella) was associated with the occurrence of this disease. We present a case of a male, 12 year old healthy patient who, five days after Infl uenza vaccination, developed erythematous papules on the trunk, abdomen and limbs, some with adherent crusts and associated systemic symptoms. This case report is notable for describing the first case of pityriasis lichenoides et varioliformis acuta associated with the vaccine against Influenza.

Pitiriasis liquenoide: presentación de un caso clínico y revisión de la literatura / Pityriasis Lichenoides: Case report and review of the literature

Introducción: La pitiriasis liquenoide es una enfermedad inflamatoria benigna de causa desconocida. Tiene dos formas de presentación: una forma aguda (PLEVA, pitiriasis liquenoide y varioliforme aguda) y otra crónica (PLC, pitiriasis liquenoide crónica). Ambas son más frecuentes en niños y adultos jóvenes. Objetivo: Describir un caso de PLC, comentar su presentación clínica, diagnóstico y tratamiento, y revisar la literatura. Caso clínico: Escolar de 7 años que presentó episodios recurrentes de lesiones tipo pápulas eritematocostrosas brillantes de distribución centrípeta, oligosintomáticas, que desaparecían dejando máculas hipopigmentadas. La biopsia de las lesiones confirmó una pitiriasis liquenoide crónica. Durante los 3 años de seguimiento se realizaron múltiples esquemas de tratamiento para atenuar las reagudizaciones, logrando una respuesta parcial. Conclusión: La PLC es una enfermedad infrecuente que representa un desafío diagnóstico y terapéutico para el médico. El diagnóstico de esta entidad se sospecha por la clínica y se confirma con la histología. No tiene tratamiento específico pero presenta buena respuesta a corticoides, antibióticos, inmunosupresores y fototerapia UVB de banda angosta (UVB-nb). Esta última es la que ha reportado los mejores resultados. Es importante el seguimiento de los pacientes por el riesgo de desarrollar enfermedades linfoproliferativas.

Introduction: Pityriasis lichenoides is a benign inflammatory disease of unknown etiology. There are two types of this condition: an acute form (PLEVA = pityriasis lichenoides et varioliformis acuta) and a chronic one (PLC = pityriasis lichenoid chronica). Both are more common in children and young adults. Objective: To describe a case of PLC, discuss its clinical presentation, diagnosis, treatment and present a review of the literature. Case Report: A seven-year-old child who presented with recurrent oligosymptomatic episodes of bright erythematous papular lesions in centripetal distribution, which subsided and left behind hypopigmented macules. The biopsy of the lesions confirmed chronic pityriasis lichenoid. During the 3 years of follow-up, multiple treatment regimens were used to reduce exacerbations, resulting in a partial response. Conclusion: PLC is a rare disease that represents a diagnostic and therapeutic challenge to the physician. The diagnosis of this condition is suspected clinically and confirmed by histology. It does not have specific treatment, but it responds well to corticosteroids, antibiotics, immunosuppressants and phototherapy with UVB narrowband (UVBnb). The latter has given the best results. It is important to monitor patients for risk of developing lymphoproliferative disorders.

Clinicopathologic analysis of 60 cases of pityriasis lichenoides et varioliformis acuta / 中华皮肤科杂志

Objective To explore the clinicopathologic features of pityriasis lichenoides et varioliformis acuta (PLEVA).Methods A retrospective analysis was performed.Clinical and histological data were collected from 60 patients with PLEVA.The clinicopathologic features of PLEVA were analyzed.Results Among the 60 patients with PLEVA,32 (53.3％) were aged 2-18 years,and 28 (46.7％) aged 19-49 years.Skin lesions were distributed in a diffuse pattern in 50 (83.3％) patients,in a central pattern in 2 (3.3％) patients,and in a peripheral pattern in 8 (13.4％) patients.Nineteen (31.6％) patients had a history of upper respiratory infection.Histopathological examination revealed liquefactive degeneration of basal cells and perivasculitis in the dermis in all the 60 cases,neutrophil abscess formation in the stratum corneum in 26 (43.3％) cases,keratinocyte necrosis in the epidermis in 41 (68.3％) cases,generalized liquefactive degeneration in 30 (50.0％) cases,migration of lymphocytes into the epidermis in 43 (71.6％) cases,Pautrier's microabscess formation in 2 cases,varying degrees of extravasation of erythrocytes into the epidermis in 46 (76.7％) cases,fibrinoid necrosis of blood vessel walls in the dermis in 3 cases.PLEVA progressed into granuloma fungoides in 1 patient.Twenty patients underwent immunohistochemical examination,and 3 of them showed monoclonal hyperplasia of T cells.Conclusions PLEVA has characteristic clinical manifestations,and the combination of pathological and clinical examination is the gold standard for its diagnosis.

Clinical and Histologic Features of Pityriasis Lichenoides: The Difference between Children and Adults / 대한피부과학회지

BACKGROUND: Pityriasis lichenoides, an inflammatory skin condition of unknown etiology affecting both children and adults encompasses a clinical spectrum between pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC). Although reported to follow a more self-limiting course in children compared with adults, there are a few studies comparing pityriasis lichenoides in both age groups in Korea. OBJECTIVE: The aim of this study was to evaluate the clinicopathological features, overall efficacy of treatments, and disease outcomes in children and adults diagnosed with pityriasis lichenoides. METHODS: This was a retrospective study of pityriasis lichenoides at our center between January 2003 and December 2013. Clinical manifestations and histopathological features of 54 patients with pityriasis lichenoides were analyzed. We also evaluated the clinical course in children and adults according to Gelmetti's classification. RESULTS: In this study, 36 patients were children (mean age 11.6 years, range 3approximately18 years) and 18 patients were adults (mean age 34.7 years, range 19approximately55 years). Lesions on the face were observed more commonly in children (n=18, 50%) compared with adults (n=1, 5.5%). Of the clinical features, vesiculation, crust and pigmentary changes were significantly more common in children than in adults. After treatment, 12 children (33.3%) and 10 adults (55.6%) went into complete remission. Histopathological features were not significantly different between children and adults. They just showed the difference between PLEVA and PLC. Dyskeratosis, epidermal necrosis, intraepidermal red blood cell (RBC) trapping, and RBC extravasati on in the dermis were more prominent in PLEVA than in PLC. By Gelmetti's classification, disease duration was longer in children with the peripheral type (38.5 months) than in children with the diffuse and central types. In adults, the diffuse type lasted longer (24.5 months) than the central and peripheral types. CONCLUSION: This study suggests that, compared with adults, pityriasis lichenoides in children is more likely to follow an unremitting course, with more facial/acral involvement and pigmentary changes and a poorer response to conventional treatment modalities. Applying the Gelmetti's classification, disease duration was longer in children with the peripheral type than other types. However, in adults, the duration was longer with the diffuse type.

Resistant Pityriasis Lichenoides et Varioloformis Acuta: Successful Treatment with Prednisolone and Methotrexate / 대한피부과학회지

No abstract available.

Pitiriasis liquenoide: estudio retrospectivo clínico epidemiológico de 49 pacientes pediátricos / Pityriasis lichenoides: a retrospective clinical and epidemiological study of 49 pediatric patients

La pitiriasis liquenoide (PL), entidad pápulo-escamosa, afecta principalmente a niños. Presenta una forma aguda, "pitiriasis liquenoide y varioliforme aguda" (PLEVA) y otra crónica, la pitiriasis liquenoide crónica (PLC). Objetivo: describir las características de pacientes con diagnóstico clínico e histopatológico de PL. Comparar nuestros resultados con los hallados en la bibliografía. Materiales y Métodos: historias clínicas e informes anátomo-patológicos de 49 pacientes que consultaron entre marzo de 2000 a marzo de 2011. Se analizaron los datos obtenidos mediante el programa SPSS 11.5. Resultados: de 49 pacientes con PL, 17 presentaron PLEVA y 32 PLC. El promedio de edad de inicio de PLEVA fue de 6 años y de 10 años para PLC. Predominó el sexo masculino en PLEVA. Las formas generalizadas parecieran ser las predominantes. Las lesiones más frecuentemente halladas fueron: pápulas y pápulas eritematosas en PLEVA, escamas en PLC y las máculas hipocrómicas como discromía residual. 14 de 49 pacientes mostraron asociaciones mórbidas. Conclusiones: la PL puede ser dividida en aguda y crónica, a pesar de existir ambas clases de lesiones en un mismo paciente. PLEVA se presentó a edades más tempranas, predominando en varones. De los pacientes que presentaron co-morbilidades, la mayoría comprometía la inmunidad.

Background: the pityriasis lichenoides (PL), a papulosquamous disease, affects mostly children. Present an acute form, pytiriasis lichenoides et varioliformis acute (PLEVA) and other chronic, chronic lichenoid pytiriasis (PLC). Objetive: to describe the caracheristics of patients with clinic and histopathological diagnosis of PL. Compare our results with those found in the literature. Materials and Methods: clinical records and pathology reports of 49 patients who consulted between March 2000 and March 2011. Data were analyzed using SPSS 11.5. Results: of 49 patients with PL, 17 presented PLEVA and 32 PLC. The average age of onset of PLEVA was 6 years and 10 years for PLC. Males predominate in PLEVA. The lesions most frequently found were: papules and erythematous papules in PLEVA, scales in PLC and hypocromic macules as residual dyschromia. 14 of 49 patients showed morbid associations. Conclusions: the PL can be divided into acute and chronic forms, despite the existence of both types of lesions in the same patient. PLEVA was presented at younger age, predominantly in males. From the patients who had co-morbidities, the most of them, compromised immunity.

Pityriasis lichenoides chronica associated with use of HMG-CoA reductase inhibitors / Pitiriasis liquenoide crónica asociada con el uso de inhibidores de la reductasa de HMG-CoA

Herein, we present three cases of Pityriasis lichenoides chronica (PLC) in patients who developed the rash after use of 3-hydroxy-3-methyl-glutaryl-Coenzyme A (HMG-CoA) reductase inhibitors. The patients had complete resolution after standard treatment by dermatologists and withdrawal of the offending agents. In one case, the patient had a previous episode of a similar rash that occurred with HMG-CoA reductase inhibitors use many years previously. Pityriasis lichenoides chronica is a condition of unknown aetiology. Several agents have been associated with its presentation. We postulate HMG-CoA reductase inhibition in skin presents a final common pathway for the presentation of PLC in select patients.

Se presentan tres casos de pitiriasis liquenoide crónica (PLC) en pacientes que desarrollaron una erupción tras el uso de inhibidores de la reductasa de la hidroxi-metilglutaril-coenzima A (HMG-CoA). Los pacientes tuvieron resolución completa después del tratamiento estándar dado por los dermatólogos, y la suspensión de los agentes ofensivos. En un caso, el paciente tuvo un episodio de una erupción similar anterior, que ocurrió debido al uso de inhibidores de la reductasa de HMG-CoA muchos años atrás. La pitiriasis liquenoide crónica es una condición de etiología desconocida. Varios agentes han sido asociados con su manifestación. Se postula que la inhibición de la reductasa de HMG-CoA presenta un camino final común para la manifestación de PLC en determinados pacientes.

Febrile Ulceronecotic Pityriasis Lichenoides et Varioliformis Acuta Successfully Treated with Oral Cyclosporin / 대한피부과학회지

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA), or febrile ulcerative Mucha-Haberman disease (FUMHD) is very rare, but potentially lethal variants of PLEVA. This subtype is characterized by rapidly progressive ulceronecrotic lesions and systemic manifestations, such as high fever, gastrointestinal, neurological, cardiologic and pulmonary involvement, and rheumatologic manifestations. Several treatments with variable response have been challenged, such as systemic steroid, antibiotics, methotrexate, dapsone, cyclosporine and ultraviolet therapy. But there is no standard therapy for FUMHD to date. We report a 59-year-old woman with FUMHD, who recurred after discontinuation of systemic steroid, but successfully treated with oral cyclosporine.

Doença de Mucha-Habermann úlceronecrótica febril em adulto com boa resposta à corticoterapia oral / Febrile ulceronecrotic Mucha-Habermann disease in adult patient successfully treated with systemic corticosteroid

A doença de Mucha-Habermann ulceronecrótica febril (FUMHD) é uma variante clínica rara da pitiríase liquenoide variceliforme aguda (PLEVA). Tem etiologia incerta e é caracterizada por lesões úlceronecróticas, associadas a sintomas sistêmicos. Relata-se um caso de paciente masculino, com início agudo de lesões máculo-papulares, vesicobolhosas e úlceronecróticas, associadas à febre alta e mialgia. Tratado com prednisona 0,5 mg/kg/dia, obteve-se excelente resposta terapêutica. A FUMHD é uma variante severa da PLEVA, cujo diagnóstico é clínico e histopatológico. Vários tratamentos são descritos, tais como: metrotexate, corticoesteroides, PUVA, mas nenhum foi estabelecido.

The Febrile Ulceronecrotic Mucha-Habermann (FUMHD) disease is a rare variant of pityriasis lichenoides et varioliformis acuta (PLEVA). Its etiology still remains unknown and it is characterized by a sudden onset of ulceronecrotic skin lesions associated with systemic symptons. It is reported here the case of a male patient with a sudden and acute evolution of macules and papules, ulceronecrotic and vesicle-bullous lesions associated with systemic symptons. The patient was treated with prednisone 0,5 mg/kg/day with a dramatic response. The FUMHD is a severe variant of PLEVA and its diagnosis is clinical and histopathological. Many treatments such as methotrexate, corticosteroids and PUVA have been described .However, none of them has been settled.

Pityriasis lichenoides with ulceronecrosis and hyperthermia: A rare variant of pityriasis lichenoides et varioliformis acuta.

Pityriasis lichenoides with ulceronecrosis and hyperthermia (PLUH) is a severe variant of pityriasis lichenoides et varioliformis acuta that is characterized by high fever and papulo-necrotic skin lesions. We report the case of a 49-year-old male with typical features of PLUH along with an unusual manifestation of extensive skin necrosis including involvement of the intertriginous regions. Systemic administration of corticosteroids and antibiotics did not help to control the disease and the patient succumbed to death due to its fulminant nature.

Doença de Mucha-Habermann (variante febril úlcero-necrótica) com acometimento mucoso exuberante: relato de caso / Febrile ulceronecrotic Mucha-Habermann disease with exuberant mucosal involvement: case report

A pitiríase liquenoide é dermatose incomum, idiopática, com espectro clínico-histopatológico onde inclui a forma varioliforme aguda (doença de Mucha-Habermann), sua variante febril úlceronecrótica e a forma crônica. Manifestações sistêmicas podem ocorrer na variante febril úlcero-necrótica, com relatos de casos fatais em adultos. Relata-se o caso de um paciente jovem, com diagnóstico clínico e histopatológico de doença de Mucha-Habermann, variante febril úlcero-necrótica, e acometimento mucoso exuberante, fato ocasional, mesmo nas formas mais graves de pitiríase liquenoide. Além do aspecto clínico inusitado, demonstra-se excelente resultado terapêutico, com a associação de prednisona e metotrexato.

Pityriasis lichenoides is a rare idiopathic cutaneous disorder, with a clinical-histopathological spectrum comprising the acute varioliform form (Mucha-Habermann's disease), its febrile ulceronecrotic variant, and its chronic form. Systemic manifestations may occur in the febrile ulceronecrotic variant, with reports of adult mortality. The case of a young male patient with clinical and histopathological diagnosis of Mucha-Habermann’s disease, febrile ulceronecrotic variant, with severe mucosal involvement - an occasional incidence even in the most severe forms of pityriasis lichenoides - is presented. In addition to the atypical clinical aspect, an excellent therapeutic result is shown with the association of prednisone and methotrexate.

Febrile Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta in a child / 대한피부과학회지

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA) is a very rare cutaneous disorder clinically characterized by the acute onset of erythematous papules, vesicles and large coalescent ulceronecrotic lesions on the whole body accompanied by high fever and severe constitutional symptoms. In general, ulceronecrotic lesion is preceded by a typical rash of PLEVA. Histopathologically, it shows widespread epidermal necrosis and fibrinoid necrosis of blood vessel walls in addition to the superficial and deep perivascular lymphocytic infiltrations. Several regimens including systemic corticosteroid, antibiotics, methotrexate or ultraviolet therapy have been challenged in this disorder . We report a typical case of febrile ulceronecrotic PLEVA in a 10-year-old child who showed a dramatic response to methotrexate.