Langerhans cell histiocytosis

Langerhans cell histiocytosis

Author Chauffaille, Maria de Lourdes Lopes Ferrari Autor UNIFESP Google Scholar
Valério, Rosana M. Autor UNIFESP Google Scholar
Diniz, Cybelle Maria Costa Autor UNIFESP Google Scholar
Simões, Milvia Maria Autor UNIFESP Google Scholar
Enokihara, Silva Autor UNIFESP Google Scholar
Michalany, Nylceo Autor UNIFESP Google Scholar
Ferreira, Karin Ventura Autor UNIFESP Google Scholar
Martinez, José Antônio Baddini Autor UNIFESP Google Scholar
Hassun, Karine Marques Autor UNIFESP Google Scholar
Atallah, Álvaro Nagib Autor UNIFESP Google Scholar
Kerbauy, José Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
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.

Os autores apresentam um caso raro de histiocitose de célula de Langerhans com apresentação lentamente progressiva, lesão vulvar, peri-anal e oral, diabete insipidus, infiltração pulmonar, dérmica e óssea em paciente de 31 anos. A imunohistoquímica da biopsia de pele foi: proteína S-100 e vimentina positivas, porém o diagnóstico definitivo foi feito pela demonstração de grânulos de Birbeck à microscopia eletrônica. O tratamento baseia-se em quimioterapia sistêmica e a lesão vulvar tem má resposta à quimioterapia.
Keywords Histiocytosis X
Langerhans cell
Diabetes insipidus
Language English
Date 1998-02-01
Published in São Paulo Medical Journal. Associação Paulista de Medicina - APM, v. 116, n. 1, p. 1625-1628, 1998.
ISSN 1516-3180 (Sherpa/Romeo)
Publisher Associação Paulista de Medicina - APM
Extent 1625-1628
Origin http://dx.doi.org/10.1590/S1516-31801998000100006
Access rights Open access Open Access
Type Article
SciELO ID S1516-31801998000100006 (statistics in SciELO)
URI http://repositorio.unifesp.br/handle/11600/575

Show full item record




File

Name: S1516-31801998000100006.pdf
Size: 729.3Kb
Format: PDF
Description:
Open file

This item appears in the following Collection(s)

Search


Browse

Statistics

My Account