A Case of Langerhans Cell Histiocytosis with Sclerosing Cholangitis.
- Author:
Jae Chul HWANG
1
;
Doh Hyun KIM
;
Eun Hee LEE
;
Jai Hak JEUNG
;
Jin Young SHIM
;
Jae Youn CHEONG
;
Do Hyun KIM
;
Kee Myung LEE
;
Byung Moo YOO
;
Jin Hong KIM
Author Information
1. Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea. jinhkim@madang.ajou.ac.kr
- Publication Type:Case Report
- Keywords:
Langerhans cell histiocytosis;
Sclerosing cholangitis;
CD1a;
S100
- MeSH:
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
- From:Korean Journal of Gastrointestinal Endoscopy
2003;26(1):56-60
- CountryRepublic of Korea
- Language:Korean
-
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.
