Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Erdheim-Chester Disease Involving the Biliary System and Mimicking Immunoglobulin G4-Related Disease: A Case Report

면역글로불린 G4 연관 질환과 유사한 담관 침범을 보이는 Erdheim-Chester 병: 증례 보고

  • Hyuk Gi Hong (Department of Radiology, Seoul Medical Center) ;
  • Yong Eun Chung (Department of Radiology, Severance Hospital, Yonsei University College of Medicine) ;
  • June Park (Department of Radiology, Severance Hospital, Yonsei University College of Medicine) ;
  • Yeo Eun Kim (Department of Radiology, Seoul Medical Center)
  • 홍혁기 (서울의료원 영상의학과) ;
  • 정용은 (연세대학교 의과대학 세브란스병원 영상의학과) ;
  • 박준 (연세대학교 의과대학 세브란스병원 영상의학과) ;
  • 김여은 (서울의료원 영상의학과)
  • Received : 2022.08.16
  • Accepted : 2022.11.13
  • Published : 2023.05.01
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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.

Erdheim-Chester 병은 희귀한 조직구증으로, 백혈구의 아형인 조직구의 과잉 생산으로 인한 질병 중 하나이며, 1930년에 처음 기술되었다. Erdheim-Chester 병은 가장 흔하게는 뼈를 침범하고, 복부의 다른 모든 기관을 침범할 수 있지만 담도 침범의 증례는 매우 드물게 보고된다. 이에 저자는 영상의학적으로 면역글로불린 G4 연관 질환과 구별하기 어려웠던 Erdheim-Chester 질환의 담도 침범 사례를 보고하고자 한다.

Keywords

References

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