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Unusual Manifestation of Immunoglobulin G4-Related Disease Involving the Upper Arm: A Case Report

상완에 발생한 면역글로불린 G4 연관 질환의 비전형적 발현: 증례 보고

  • Jin Hee Park (Department of Radiology, College of Medicine, Inje University, Busan Paik Hospital) ;
  • Sun Joo Lee (Department of Radiology, College of Medicine, Inje University, Busan Paik Hospital) ;
  • Hye Jung Choo (Department of Radiology, College of Medicine, Inje University, Busan Paik Hospital)
  • 박진희 (인제대학교 의과대학 인제대학교 부산백병원 영상의학과) ;
  • 이선주 (인제대학교 의과대학 인제대학교 부산백병원 영상의학과) ;
  • 추혜정 (인제대학교 의과대학 인제대학교 부산백병원 영상의학과)
  • Received : 2022.02.22
  • Accepted : 2022.07.11
  • Published : 2023.05.01

Abstract

Immunoglobulin G4 (IgG4)-related disease is a rare systemic fibroinflammatory condition characterized by organomegaly or tumefactive lesions associated with lymphoplasmacytic infiltration rich in IgG4 plasma cells. We report a case of IgG4-related disease involving the subcutaneous layer of the left upper arm in a 48-year-old female presenting with an unusual soft tissue mass. US and MRI showed an irregular infiltrative soft tissue mass, indicating possible malignancy or inflammation. We discuss the diagnostic criteria, histopathologic features, radiological features, and treatment of IgG4-related disease.

면역글로불린 G4(immunoglobulin G4; 이하 IgG4) 관련 질환은 드문 전신성 섬유염증 상태로 IgG4 형질 세포가 풍부한 림프구 침윤과 관련된 기관 비대 또는 종창성 병변을 특징으로 한다. 우리는 48세 여자 환자에서 드문 소견인 연조직 종괴로 발현된 왼팔의 피하층을 침범한 IgG4 관련 질환 증례를 보고하고자 한다. 초음파 및 자기공명영상을 촬영하였으며 악성 또는 염증 병변이 의심되는 불규칙하며 침윤성 경계를 갖는 연조직 종괴가 관찰되었다. 우리는 IgG4 관련 질환의 진단 기준, 조직 병리학적 특징, 영상의학적 특징, 및 치료 방법에 대해 논의하고자 한다.

Keywords

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