A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis

Vijayvardhan Kamalumpundi; John Rieth; Huy Tran; Munish Ashat; Xiaocen Zhang

doi:10.14309/crj.0000000000000963

A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis

ACG Case Rep J. 2023 Jan 20;10(1):e00963. doi: 10.14309/crj.0000000000000963. eCollection 2023 Jan.

Authors

Vijayvardhan Kamalumpundi¹, John Rieth², Huy Tran³, Munish Ashat³, Xiaocen Zhang³

Affiliations

¹ Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA.
² Division of Hematology, Oncology, and Blood & Marrow Transplantation, University of Iowa Hospitals and Clinics, Iowa City, IA.
³ Department of Internal Medicine, Division of Gastroenterology and Hepatology, University of Iowa Hospitals and Clinics, Iowa City, IA.

Abstract

Erdheim-Chester disease (ECD) is an exceedingly rare and aggressive disease characterized by foamy CD68 + CDa-histiocytic infiltration into multiple tissues and organs. Only 1,500 cases have been diagnosed since 1930 when ECD was first described. Biliary tract involvement of ECD has only been reported in the literature once. We report a case of ECD causing extrahepatic biliary obstruction without significant bile duct dilation, mimicking primary sclerosing cholangitis or IgG4 disease.

Keywords: IgG4 disease; histiocytosis; myeloid neoplasms; obstructive cholangiopathy.

Publication types

Case Reports