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 vimentin, but the diagnosis was done with the demonstration of Birbeck granules with electronic microscopy. The treatment was based on systematical chemotherapy although vulvar lesion has a bad response to chemotherapy.
Highlights
The authors present a rare case of Langerhans cell histiocytosis (LCH) with vulvar, perianal and oral lesions, pulmonary and diabetes insipidus(DI), skin and bone infiltrations
Chauffaille Rua Botucatu, 740 - 3Q andar Sao Paulo/SP - Brasil - CEP 04023-900 in the vul va that had appeared 1 year previously. She complained of cutaneous nodes in the right shoulder and forearm, gengivitis, loosening of the teeth, halitosis, otorrhea, polydipsia, amenorrhea, galactorrhea and a weight gain of 24 kg in the three previous years, as well as a spontaneous pneumothorax 10 years earlier
Laboratory exams: Hb=13.7g/dl; WBC= 6.700/mm[3]; Platelets=230.000/ mm[3]; SGOT=54(13), SGPT=60(14), alkaline phosphatase =340(250), LDH=424(450), uric acid=7.8 (6.0); the pulmonary function had a mixed ventilatory abnormality and predominance of a light restrictive pattern; transbronchial biopsy presented unspecific infiltrate; water deprivation test consistent with DI and megatest consistent
Summary
Disciplina de Hematologia e Hemoterapia, Disciplina de Clinica Medica, Disciplina de Anatomia Patologica, Disciplina de Dermatologia, Disciplina de Pneumologia - Escola Paulista de Medic in a, Universidade Federal de Siio Paulo - Siio Paulo, Brazil. 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 S 100 protein and vimentine, but the diagnosis was done with the demonstration of Birbeck granules with eletronic mucroscopy. The treatment was based on systemical chemotherapy vulvar lesion has a bad response to chemotherapy.
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