RESUMO
INTRODUCTION: Langerhans cell histiocytosis (LCH) is a rare disease that mainly affects young children. Sclerosing cholangitis may occur in 10-15% of patients with the multivisceral form. We report the case of a 15-month-old child who presented sclerosing cholangitis revealing LCH. OBSERVATION: A 15-month-old child was hospitalized for cholestatic jaundice. He was the son of consanguineous parents and had repeated ear infections. One month before his hospitalization, he developed febrile jaundice. Initial clinical examination showed hepatosplenomegaly, with cholestasis, bicytopenia, and biological inflammatory syndrome. The digestive radiological studies revealed hepatomegaly and a regular thickening of the intestinal wall with an extension to the biliary tree. During his hospitalization, the infant developed stubborn ascites, lymphadenopathy, and skin lesions. Skull radiographs revealed punched-out lesions. The skin biopsy confirmed the diagnosis of histiocytosis X. Chemotherapy was started. The child died after the first course of treatment as a consequence of liver failure. CONCLUSION: Sclerosing cholangitis may complicate LCH, mainly in its multivisceral form. On average, sclerosing cholangitis develops 2 years after diagnosis in children. It is rarely indicative of the diagnosis, which is mainly based on radiological examinations. Liver involvement is a factor of poor prognosis. It precipitates the occurrence of biliary cirrhosis. Usually, sclerosing cholangitis responds poorly to Langerhans histiocytosis treatment and liver transplantation must be considered.
