Langerhans cell histiocytosis

• Main Authors: Maria de Lourdes L. F. Chauffaille, Rosana M. Valério, Cybelle Maria Costa Diniz, Milvia Maria Simões, Silva Enokihara, Nylceo Michalany, Karin Ventura Ferreira, José Antonio Baddini Martinez, Karine Marques Hassun, Álvaro Nagib Atallah, José Kerbauy
• Format: Article
• Language: English
• Published: Associação Paulista de Medicina
• Series: São Paulo Medical Journal
• Subjects: Histiocytosis X; Langerhans cell; Diabetes insipidus
• Online Access: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801998000100006&lng=en&tlng=en

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.