Korean J Gastrointest Endosc.  2003 Jan;26(1):56-60.

A Case of Langerhans Cell Histiocytosis with Sclerosing Cholangitis

• Hwang JC
• Kim DH
• Lee EH
• Jeung JH
• Shim JY
• Cheong JY
• Kim DH
• Lee KM
• Yoo BM
• Kim JH

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.

Keyword

Langerhans cell histiocytosis; Sclerosing cholangitis; CD1a; S100

MeSH Terms

Bile Ducts
Bile Ducts, Extrahepatic
Biopsy
Cholangiopancreatography, Endoscopic Retrograde
Cholangitis, Sclerosing*
Common Bile Duct
Constriction, Pathologic
Dilatation
Eosinophils
Fibrosis
Histiocytes
Histiocytosis, Langerhans-Cell*
Humans
Keratins
Mucous Membrane
Keratins