RESUMEN
A histiocitose de células de Langerhans corresponde a um grupo heterogêneo de desordens caracterizadas pela proliferação monoclonal de células dendríticas. Predomina na infância e pode afetar qualquer órgão. Relata-se caso de paciente, sexo feminino, 44 anos, apresentando placas espessas, exsudativas, com escamas aderentes aos pelos, localizadas no couro cabeludo, semelhantes a dermatite seborreica, além de fístulas nas axilas, regiões inframamárias e inguinais de evolução há 16 anos. Realizou-se biópsia da lesão cutânea seguida de imunohistoquímica que concluiu diagnóstico de Histiocitose de células de Langerhans. Investigação sistêmica evidenciou acometimento pulmonar concomitante. Até o presente momento existem poucas publicações sobre envolvimento cutâneo em adultos, assim como não há protocolos de tratamento para os mesmos, necessitando maiores estudos para melhor manejo desses pacientes.
Langerhans cell histiocytosis corresponds a heterogeneous group of disorders characterized by monoclonal dendritic cells proliferation, it predominates in childhood which may affect any organ of the body. The case reports of a female patient, aged 44, presenting thick plates with scales adhering to the hairs, scalp located, similar to seborrheic dermatitis, besides fistulas in axillas, inguinal and infra mammary regions. The hypothesis of Langerhans cell histiocytosis was confirmed by cutaneous biopsy and immunohistochemistry. Systemical investigation accused pulmonary involvement. Until now are few publications about adult cutaneous cases, so none treatment protocols are avaible for them. More specific studies are demanded for better management of these patients.
Asunto(s)
Histiocitosis , Histiocitosis de Células de Langerhans , Cuero Cabelludo , Células Dendríticas , Dermatitis Seborreica , Antígenos CD1RESUMEN
Chronic mucocutaneous candidiasis is a rare disorder characterized by persistent and recurrent infections by Candida due to changes in cellular immunity and may be associated with autoimmune endocrine disorders. It is refractory to the usual antifungal treatments, which merely control it with imidazole derivatives. This reports the case of a 50-year-old female patient who referred vaginal discharge associated with vulvar ulcerated lesions and whitish plaques on oral and genital mucous membranes of onset in adolescence besides cutaneous horns in nipples. The clinical picture, family history, culture and anatomopathological studies were consistent with chronic infection by candida. Treatment with systemic antifungals obtained partial response of lesions characterizing a clinical picture of Chronic Mucocutaneous Candidiasis.