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A Case of POEMS Syndrome Associated with Sarcoidosis

유육종증을 동반한 POEMS 증후군 1예

  • Yoon, Hwan (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kang, Byung-Ju (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Woo-Sung (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Dong-Soon (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Song, Jin-Woo (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine)
  • 윤환 (울산대학교 의과대학 서울아산병원 내과) ;
  • 강병주 (울산대학교 의과대학 서울아산병원 내과) ;
  • 김우성 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 김동순 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 송진우 (울산대학교 의과대학 서울아산병원 호흡기내과)
  • Published : 2012.07.01

Abstract

POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Pulmonary manifestations of POEMS syndrome are common, with frequent symptomatic and asymptomatic respiratory involvement on presentation. However, there have been no reported cases of concomitant sarcoidosis and POEMS syndrome. We treated a 55-year-old woman who presented with dyspnea and bilateral lower leg pitting edema. She had been diagnosed with sarcoidosis 4 years earlier. We reassessed the patient meticulously and found polyneuropathy, IgG lambda type monoclonal gammopathy, lymphadenopathy, hepatosplenomegaly, hypothyroidism, hypertrichosis, and pleural and pericardial effusions, all of which led us to diagnose POEMS syndrome. Immunosuppression with high-dose dexamethasone led to clinical and radiological improvement.

POEMS 증후군은 다발성신경병증(polyneuropathy), 장기종대(organomegaly), 다양한 형태의 내분비장애(endocrinopathy), 단클론성 감마글로블린병증(monoclonal gammopahty), 피부병변(skin lesion)을 특징으로 하는 드문 질환으로, 폐침범의 형태로 유육종증을 동반한 예는 보고된 바가 없다. 저자들은 유육종증(sarcoidosis)을 동반한 POEMS 증후군 환자를 경험하였기에 보고하는 바이다.

Keywords

References

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