A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis

A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis

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 report...

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Veröffentlicht in:ACG case reports journal 2023-01, Vol.10 (1), p.e00963-e00963
Hauptverfasser: Kamalumpundi, Vijayvardhan, Rieth, John, Tran, Huy, Ashat, Munish, Zhang, Xiaocen
Format: Artikel
Sprache:eng
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Case Report
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Zusammenfassung: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.
ISSN:2326-3253
2326-3253
DOI:10.14309/crj.0000000000000963