Histiocitoses de células de Langerhans no Paciente Pediátrico: apresentação de um caso clínico

Abstract

In the pediatric group histiocytoses are rare diseases with diverse and severe staff in its clinical presentation. To describe a case of Langerhans cell histiocytosis in childhood, more specifically syndromic Letterer-Siwe disease. Case report: Infant, 7 months, male, blotches frame erythematous and pruritic, febrile periods, bulging region BTE bilaterally fibroelastic consistency, fetid otorrhea left, nail lesions and ulcers in ophthalmic mucosa, hepatosplenomegaly, skin jaundice and sclera, tumor increase retroauricular bilaterally evluindo with pnmeumonia worsening hepatic framework cuminando with death. CT chest bilateral sparse pulmonary cysts accompanied by multiple lymph node enlargement mediastinal coalescing. A CT scan of the abdomen hepatomegaly with multiple nodules. Magnetic Resonance Cholangiopancreatography suggestive of congenital hepatic fibrosis associated with fibrocystic changes, saccular dilation. Immunohistochemical evaluation of cervical biopsy was positive for anti-CD68 antibodies, CD1a and S100, and negative for CD30, concluding diagnosis of Langerhans cell histiocytosis, Letterer-Siwe disease. There was no Langerhans cells to liver biopsy. The diagnosis of LCH is a challenge to the pediatrician that requires precision and speed to initiation of therapy, and should be remembered as a differential diagnosis, despite its rare incidence. Keywords: Histiocytosis; Letterer-Siwe Disease; Hepatosplenomegaly; Salivary Gland; Pediatrics.