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A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis
Journal article   Open access   Peer reviewed

A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis

Vijayvardhan Kamalumpundi, John Rieth, Huy Tran, Munish Ashat and Xiaocen Zhang
ACG case reports journal, Vol.10(1), e00963
01/20/2023
DOI: 10.14309/crj.0000000000000963
PMCID: PMC9857465
PMID: 36699184
url
https://doi.org/10.14309/crj.0000000000000963View
Published (Version of record) Open Access

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.
Case Report histiocytosis IgG4 disease Liver myeloid neoplasms obstructive cholangiopathy

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