RESUMO
Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.
Assuntos
Histiocitose de Células de Langerhans/diagnóstico , Cavidade Nasal/anormalidades , Fístula Bucal/diagnóstico , Corticosteroides/administração & dosagem , Adulto , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/tratamento farmacológico , Humanos , Masculino , Cavidade Nasal/cirurgia , Fístula Bucal/etiologia , Fístula Bucal/cirurgia , Vimblastina/administração & dosagemRESUMO
The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca syndrome. Biological findings showed an inflammatory syndrome, renal failure, proteinuria (1g / 24h), positive auto antibodies and anti DNA. Lung radiology revealed medistinal lymphadenopathy, pleural nodules, ground glass infiltrates and pleuritis. Bronchial biopsy showed non specific inflammation. The salivary gland biopsy showed amyloïd deposits. This case report reminds us that lupus and Amyloïdosis association, although exceptional remains possible. The occurrence of Lofgren syndrome in this situation make the originality of this report.