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.

Manifestaciones atípicas del síndrome de vasculitis-angeítis- endotelitis (SVAE) cutánea / Atypical manifestations of cutaneous vasculitis- angeitis-endotheliitis syndrome

Se reportan dos pacientes con diagnóstico histopatológico de vasculitis cutánea de presentación clínica atípica. Un joven de 13 años con lesiones bullosas y flictenulares generalizadas de 2-3 cm sin sobreinfección, que se iniciaron en la cavidad oral y se extendieron al tórax anterior y extremidades, asociadas con infección ventilo-respiratoria tipo neumonía adquirida en la comunidad (NAC), que requirió antibióticos y corticoides sistémicos, con resolución completa a los 17 días y diagnóstico definitivo de vasculitis por hipersensibilidad. El otro paciente corresponde a un adulto de 58 años con historia de 18 meses de lesiones maculares purpúricas en miembros superiores e inferiores, algunas confluyen generando placas violáceas no mayores de 5 cm. Cursó con diabetes, retinopatía e hipertensión arterial. La biopsia mostró vasculitis leucocitoclásica con "signo de promontorio", por lo cual se sospechó sarcoma de Kaposi que se descartó. El diagnóstico definitivo fue vasculitis linfocítica en pitiriasis liquenoide crónica (PLC).

Two patients with histopathological diagnosis of cutaneous vasculitis of atypical clinical course are presented. The first patient was a 13-year-old boy with bullous lesions and generalized phlyctenules measuring 2-3 cm with no superinfection, which began in the oral cavity and extended to the anterior chest and extremities, associated with ventilation-respiratory community acquired pneumonia (CAP), which required antibiotic therapy and systemic corticoids, with complete resolution at 17 days and definitive diagnosis of hypersensitivity vasculitis. The other patient corresponds to a 58-year-old man with purpuric macular lesions in upper and lower extremities of 18 months duration. Some of them were confluent purpuric plaques not greater than 5 cm. Patient had associated diabetes, retinopathy and hypertension. Biopsy showed leukocytoclastic vasculitis with the presence of "promontory sign" suggestive of Kaposi ́s sarcoma which was ruled out. The definitive diagnosis was lymphocytic vasculitis in a patient with pytriasis lichenoids chronica (PLC).

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.

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.

Therapeutic Response and Adverse Effects of Methotrexate in Korean Patients with Dermatological Diseases / 대한피부과학회지

BACKGROUND: Methotrexate (MTX) is a synthetic folic acid analogue with anti-proliferative and anti-inflammatory properties, and is prescribed for a variety of dermatological disorders. OBJECTIVE: The aim of this study was to investigate the therapeutic responses, adverse effects, and risk factors of MTX for dermatological use. METHODS: We retrospectively evaluated age, gender, underlying diseases, initial and cumulative doses of MTX, treatment duration, physician's global assessment (PGA) scores, and adverse effects in 200 dermatological patients treated with MTX. RESULTS: Various dermatoses responded to MTX in the following order of effectiveness: psoriasis vulgaris, pustular psoriasis, pityriasis lichenoides, atopic dermatitis, mycosis fungoides, and morphea. Adverse effects of MTX were observed in 64 of the 200 patients (32%) as follows: elevated liver function tests (LFTs) in 36 (18%), nausea or vomiting in 22 (11%), complete blood count abnormalities in 5 (2.5%), and a burning sensation over the lesion in 4 (2%). All patients who showed abnormal LFTs were normalized after reducing or stopping MTX, and the mean time to normalization was 5.76 weeks. Liver fibrosis was not found. Risk factors for MTX-related adverse effects were old age (p=0.028), skin disease (p=0.018), high initial dose of MTX (p=0.023), and high cumulative dose of MTX (p=0.044). CONCLUSION: Among various dermatoses, psoriasis showed an excellent response to MTX with relatively acceptable safety in Koreans, but regular monitoring of MTX-related adverse effects is important. The risk factors for MTX-related adverse effects were old age, psoriasis, high initial dose, and high cumulative dose.

Doença de Mucha-Habermann / Mucha-Habermann disease

Os autores descrevem um caso de doença de Mucha-Habermann (DMH), que cursou com quadro sugestivo de síndrome de ativação macrofágica (SAM). O objetivo do trabalho foi descrever um caso de rara vasculite de Mucha-Habermann (pitiríase liquenoide e varioliforme aguda - PLEVA) em paciente de 28 anos que apresentou lesões ulceronecróticas generalizadas em pele e mucosas, acometimento gastrointestinal, cardíaco e hepático, associados a febre alta contínua, com provável evolução para SAM e posterior óbito. Trata-se de doença rara, potencialmente fatal, com graves complicações sistêmicas. Os autores ressaltam a importância de seu diagnóstico e de tratamento agressivo.

A case of Mucha-Habermann disease (MHD), possibly associated with macrophage activation syndrome (MAS), is reported. The purpose of this paper was to describe the rare MHD (also known as pityriasis lichenoides et varioliformis acuta - PLEVA) in a 28-year-old male, who presented with generalized ulceronecrotic lesions on the skin and mucosae, gastrointestinal involvement, and heart andliver failure, associated with continuous high fever.The patient might have progressed to MAS and eventually died. The MHD is rare, potentially fatal and has severe systemic complications.The importance of early diagnosis and aggressive treatment is emphasized.

Haga su diagnóstico / Make your diagnosis

El diagnóstico es Pitiriasis liquenoide crónica

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.

Pitiriasis liquenoide: descripción de un caso / Pityriasis lichenoides: description of a case

La pitiriasis liquenoide (PL) es una dermatosis inflamatoria adquirida, idiopática, infrecuente, benigna y autoinvolutiva, que tiene dos variantes: aguda y crónica, con base en las diferencias morfológicas, la evolución temporal y el curso de este trastorno. Éstas pueden considerarse como los dos extremos clínicos dentro del espectro de una misma enfermedad. Clínicamente la PL se caracteriza por el desarrollo de brotes recurrentes de pápulas eritemato-descamativas liquenoides, de superficie queratósica, que pueden persistir por semanas o meses (crónica), o bien brotar y recidivar con exacerbaciones agudas acompañadas, frecuentemente, por vésico-pústulas, ulceración, hemorragias y costras (aguda), que curan dejando cicatrices atróficas varioliformes. Presentamos el caso de un escolar de 9 años de edad con una PL con escasas costras hemáticas puntiformes y con máculas hipopigmentadas residuales.

Pityriasis lichenoides (PL) is an inflammatory acquired idiopathic, rare, benign and self-healing dermatosis, which has two variants: acute and chronic, based on morphological differences, evolution and course of the disorder that can be regarded as clinical extremes within the spectrum of the same disease. It is characterized by the development of recurrent outbreaks of scaly erythematous lichenoid papules, with keratotic surface, which can persist for weeks or months (chronic) or sprout with acute exacerbations frequently accompanied by vesico-pustules, ulceration, bleeding and crusting (acute) that heal leaving atrophic varioliformis scars. We report the case of a 9 years old boy who developed pityriasis lichenoides with few punctate hematic crusts and residual hypopigmented macules.

Enfermedad de Mucha Habermann asociada a artritis reumatoide / Mucha Habermann disease associated with a rheumatoid arthritis

La pitiriasis liquenoide varioliforme aguda o enfermedad de Mucha Habermann es una afección cutánea rara de etiología no precisada, probablemente autoinmune, caracterizada por lesiones papulares de aparición aguda, con formación de pseudovesículas que sufren necrosis central, a veces con tendencia a formar costras. Se presentó el caso de una mujer de 36 años, con antecedentes de artritis reumatoidea, que ingresa por erupción generalizada en piel con formación de vesículas sobre fondo eritematoso, algunas coalescentes, con costras, prurito moderado y fiebre de hasta 39,2 °C, a la que se realiza biopsia de piel que confirma este diagnóstico, evolutivamente mejoran las lesiones en piel pero presenta infartos digitales propios de vasculitis de pequeños vasos, que apoya la controversial hipótesis del origen vasculítico. La paciente evolucionó favorablemente con tratamiento sintomático, por tener la enfermedad un curso autolimitado y generalmente benigno

Acute varioliform lichenoides pityriasis or Mucha Haberman disease is a rare cutaneous affection of unknown etiology, probably autoimmune, characterized by popular lesions of acute appearance with formation of pseudovesicles with central necrosis, sometimes with a trend to crusts. This is the case of a woman aged 36 with a history of rheumatoid arthritis admitted due to a cutaneous systemic eruption with formation of vesicles over erythematous arrangement, some underwent coalescence with crusts, a moderate pruritus and fever up to 39.2 °C; skin biopsy confirmed this diagnosis, skin lesions improve in a evolutionary way but with digital infarction typical of small vessels vasculitis supporting the polemic hypothesis of vascular origin. Patient evolved favourably with symptomatic treatment because of the disease has a self-limited and generally benign course

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.