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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Authors: 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]
Format: Article
Status:Published version
Publication date:1998
Country:Brasil
Institution:UNIFESP
Repository:Repositório Institucional da UNIFESP
Language:English
OAI Identifier:oai:repositorio.unifesp.br:11600/575
Online Access:https://dx.doi.org/10.1590/S1516-31801998000100006
https://repositorio.unifesp.br/handle/11600/575
Access Level:Open access
Keyword:Histiocytosis X
Langerhans cell
Diabetes insipidus
Summary: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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