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Gangrenous Cryoglobulinemic Vasculitis in a Patient with Multiple Myeloma

다발골수종에 동반된 괴사성 한랭글로불린혈관염 1예

  • Ryu, Hyewon (Department of Internal Medicine, Chungnam National University School of Medicine) ;
  • Park, Bomi (Department of Internal Medicine, Chungnam National University School of Medicine) ;
  • Moon, Ji Young (Department of Internal Medicine, Chungnam National University School of Medicine) ;
  • Lee, Myung-Won (Department of Internal Medicine, Chungnam National University School of Medicine) ;
  • Choi, Yoon-Seok (Department of Internal Medicine, Chungnam National University School of Medicine) ;
  • Song, Ik-Chan (Department of Internal Medicine, Chungnam National University School of Medicine) ;
  • Jo, Deog-Yeon (Department of Internal Medicine, Chungnam National University School of Medicine)
  • 류혜원 (충남대학교 의과대학 내과학교실) ;
  • 박보미 (충남대학교 의과대학 내과학교실) ;
  • 문지영 (충남대학교 의과대학 내과학교실) ;
  • 이명원 (충남대학교 의과대학 내과학교실) ;
  • 최윤석 (충남대학교 의과대학 내과학교실) ;
  • 송익찬 (충남대학교 의과대학 내과학교실) ;
  • 조덕연 (충남대학교 의과대학 내과학교실)
  • Received : 2013.04.29
  • Accepted : 2013.07.19
  • Published : 2013.12.01

Abstract

We report a 62-year-old woman with multiple myeloma associated with cryoglobulinemia accompanied by gangrene of the digits. She presented with generalized purplish net-like discoloration (livedo reticularis), which was more prominent in the lower extremities. Multiple small shallow ulcers with crusts were found in places. In addition, gangrene was observed in both ear helices, both index fingers, and several toes. The patient had monoclonal gammopathy consisting of IgG and kappa (3.95 g/dL), cryoglobulinemia, and bone marrow plasmacytosis (42%). A biopsy of a discolored skin patch on the lower leg revealed leukocytoclastic vasculitis. She was diagnosed with multiple myeloma associated with cryoglobulinemia. Immediate plasmapheresis halted the progression of the skin lesions and digital gangrene. Two cycles of thalidomide plus dexamethasone therapy led to a partial response. This case highlights the need to search for cryoglobulinemia and multiple myeloma when we see livedo reticularis or multiple skin ulcers with obscure causes.

다발골수종에 한랭글로불린혈증이 동반되는 경우는 매우 드물다. 저자들은 다발 피부 궤양과 말단부의 괴사로 내원하여 다발골수종과 한랭글로불린혈증으로 진단하고 혈장교환술과 thalidomide와 dexamethasone으로 잘 치료된 증례를 경험하였다. 본 증례는 원인 불명의 사지와 말단부의 자반성 또는 궤양성 피부병변이 있는 환자에서 드물기는 하지만 한랭글로불린혈증, 더 나아가는 다발골수종의 가능성을 배제할 필요가 있다는 것과 다발골수종 환자에서 급격하게 진행하는 한랭글불린혈증의 증상을 혈장교환술을 통해 완화시킬 수 있다는 것을 시사한다.

Keywords

References

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