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Erdheim-Chester Disease Involving the Biliary System and Mimicking Immunoglobulin G4-Related Disease: A Case Report

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Specialty Radiology
Date 2023 Jun 16
PMID 37324994
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Abstract

First described in 1930 as a lipoid granulomatosis, Erdheim-Chester disease (ECD) is a rare histiocytosis encompassing a group of disorders caused by overproduction of histiocytes, a subtype of white blood cells. This disease most commonly involves the bones and can affect organs in the abdomen; however, biliary involvement is rarely reported. We report a case of ECD with biliary involvement, which rendered it difficult to radiologically distinguish ECD from immunoglobulin G4-related disease.

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