Langerhans cell histiocytosis

The authors present a rare case of Langerhans cell histiocytosis in a 31 year old female patient with vulvar, peri-anal and oral lesions, diabetes insipidus, pulmonary skin and bone infiltrations. Skin biopsy immunohistochemistry presented positive S100 protein and vimentine, but the diagnosis was d...

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Detalhes bibliográficos
Autores: Chauffaille, Maria de Lourdes Lopes Ferrari [UNIFESP], Valério, Rosana Maria [UNIFESP], Diniz, Cybelle Maria Costa [UNIFESP], Enokihara, Mílvia Maria Simões e Silva [UNIFESP], Enokihara, Silva [UNIFESP], Michalany, Nylceo [UNIFESP], Ferreira, Karin Ventura [UNIFESP], Martinez, José Antônio Baddini [UNIFESP], Hassun, Karine Marques [UNIFESP], Atallah, Álvaro Nagib [UNIFESP], Kerbauy, José [UNIFESP]
Formato: artículo
Estado:Versión publicada
Fecha de publicación:1998
País:Brasil
Recursos:Universidade Federal de São Paulo (UNIFESP)
Repositorio:Repositório Institucional da UNIFESP
Idioma:inglés
OAI Identifier:oai:repositorio.unifesp.br:11600/575
Acesso em linha:https://dx.doi.org/10.1590/S1516-31801998000100006
https://repositorio.unifesp.br/handle/11600/575
Access Level:acceso abierto
Palavra-chave:Histiocytosis X
Langerhans cell
Diabetes insipidus
Descrição
Resumo:The authors present a rare case of Langerhans cell histiocytosis in a 31 year old female patient with vulvar, peri-anal and oral lesions, diabetes insipidus, pulmonary skin and bone infiltrations. Skin biopsy immunohistochemistry presented positive S100 protein and vimentine, but the diagnosis was done with the demonstration of Birbeck granules with eletronic mucroscopy. The treatment was based on systemical chemotherapy although vulvar lesion has a bad response to chemotherapy.

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