Langerhans cell histiocytosis

Author:

Chauffaille Maria de Lourdes L. F.1,Valério Rosana M.1,Diniz Cybelle Maria Costa1,Simões Milvia Maria1,Enokihara Silva1,Michalany Nylceo1,Ferreira Karin Ventura1,Martinez José Antonio Baddini1,Hassun Karine Marques1,Atallah Álvaro Nagib1,Kerbauy José1

Affiliation:

1. Universidade Federal de São Paulo, Brazil

Abstract

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.

Publisher

FapUNIFESP (SciELO)

Subject

General Medicine

Reference4 articles.

1. Case Report: reversible hepatomegaly and diabetes mellitus in an adult with disseminated histiocytosis X;Trochtenberg DS;Am J Med Sci,1990

2. Pulmonary Langerhans cell granulomatosis (histiocytosis X);Soler P;Annu Rev Med,1992

3. Langerhans cell histiocytosis of the female genital tract;Axiotis CA;Cancer,1991

4. Introduction: the histiocytic syndromes;Komp DM;Sem in Oncol,1991

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