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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Detalles Bibliográficos
Autores: Chauffaille, Maria de Lourdes L. F., Valério, Rosana M., Diniz, Cybelle Maria Costa, Simões, Milvia Maria, Enokihara, Silva, Michalany, Nylceo, Ferreira, Karin Ventura, Martinez, José Antonio Baddini, Hassun, Karine Marques, Atallah, Álvaro Nagib, Kerbauy, José
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:1998
País:Brasil
Institución:Associação Paulista de Medicina
Repositorio:São Paulo medical journal (Online)
Idioma:inglés
OAI Identifier:oai:ojs.diagnosticoetratamento.emnuvens.com.br:article/2197
Acceso en línea:https://periodicosapm.emnuvens.com.br/spmj/article/view/2197
Access Level:acceso abierto
Palabra clave:Histiocytosis X
Langerhans cell
Diabetes insipidus
Descripción
Sumario: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.