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 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.

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.

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.

Avaliação da expressão das citoqueratinas no líquen plano e na displasia liquenóide / Assessment of cytokeratin expression in lichen planus and lichenoid dysplasia

A avaliação da expressão das citoqueratinas (CKs) pela técnica de imunoistoquímica é muito utilizada tanto no auxílio diagnóstico de vários tipos de lesões, com também no seu prognóstico. O líquen plano e a displasia liquenóide são lesões que apresentam várias características clínicas e histológicas em comum, perpetuando na literatura a discussão a respeito do real potencial maligno do líquen plano. Nosso trabalho teve por objetivo avaliar a expressão das CKs 7, 10, 13, 14, 16 e 19 pela técnica de imunoistoquímica, através do método da estreptavidina-biotina, em cortes de líquen plano e displasia liquenóide, utilizando mucosa normal como controle. Os resultados demonstraram que não houve alterações significativas no padrão de marcação entre as lesões, não tendo sido identificado nenhum tipo de CK específica que possa ser utilizada para diferenciá-las. Dessa forma, podemos concluir que o exame pela coloração com hematoxilina e eosina deve ainda ser, em conjunto com as informações clínicas dos casos, ser soberano na avaliação dessas lesões, devendo a avaliação da expressão das CKs ser considerada como um método de auxílio diagnóstico

Pityriasis lichenoides et varioliformis acuta in pregnancy

Pityriasis lichenoides et varioliformis acuta is an uncommon disease, especially during pregnancy. In review of the obstetric literature, there was no report of pityriasis lichenoides et varioliformis acuta during pregnancy. A 25-year-old female was seen at 24 weeks of gestation for consultation about a cutaneous disease. She was admitted at 30 weeks of gestation because of threatened premature labor, and some active cutaneous papules presented themselves at that time. After the treatment, cutaneous papules remitted. But at 35 weeks of gestation, she had spontaneous labor. Both the mother and infant were doing well at 5 months postpartum. If pityriasis lichenoides et varioliformis acuta exists in the vagina or cervical bone of the uterus, it is due to infections from lymphatic vasculitis and necrosis. It may cause threatened premature labor and premature rupture of the membrane