A Case of Langerhans Cell Histiocytosis with Sclerosing Cholangitis / 대한소화기내시경학회지

ABSTRACT

Langerhans cell histiocytosis is a rare disorder with abnormal proliferation of histiocytes. Besides the infiltration of a variety of organs, patients with Langerhans cell histiocytosis can develop sclerosing cholangitis, with bile duct involvement, progressive fibrosis, and cirrhosis. We report a case of Langerhans cell histiocytosis with sclerosing cholangitis. Endoscopic retrograde cholangiopancreatography revealed multiple strictures and dilatations of left intrahepatic and extrahepatic bile duct. Endoscopic biopsy of common bile duct showed diffuse infiltration of histiocytes and eosinophiles in lamina propria. Immunohistochemical stain showed positive reactions for S-100 and CD1a and a negative reaction for cytokeratin in infiltrating histiocytes.