A 43 year-old man with a 15-year history of disseminated Langerhans histiocytosis (LH) in complete clinical remission was admitted with jaundice and abdominal pain. Pathological examination demonstrated LH in the choledochus associated with sclerosing cholangitis in the liver. Immunohistochemistry for S-100 protein and electron microscopy of the choledochus tissue showed Langerhans cell-like elements in the infiltrate. Our findings suggest that, in patients with disseminated Langerhans histiocytosis, liver function should be monitored, particularly, for signs of biliary obstruction, and demonstrate that such a finding is possible in adults.
