POEMS syndrome with extensive calciphylaxis: A case report and literature review

Cheng Han Liu, Yu Chen Chen, Tsai Yun Chen, Hang Wei Huang, Julia Lee

Research output: Contribution to journalArticlepeer-review

Abstract

POEMS syndrome, a paraneoplastic disorder, caused by plasma cell dyscrasia, is characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. Calciphylaxis is a vasculopathy of subcutaneous arteries with calcification and skin ulceration, most commonly associated with end-stage renal disease. Calciphylaxis occurring in patients with POEMS syndrome is very rare; only 8 cases have been reported to date. We report the 9thcase. A middle-aged woman with chronic inflammatory demyelinating polyneuropathy and monoclonal plasma cell proliferative disorder with IgA gammopathy presented with a 3-month history of multiple painful violaceous plaques with gangrenous necrosis and ulceration on the abdomen and extremities. The skin lesions worsened after surgical debridement and fasciotomy but improved with weekly intravenous bortezomib combined with thalidomide and dexamethasone for POEMS syndrome. The present case illustrates that effective control of the disease activity of POEMS syndrome may improve the surgical outcome of extensive skin necrosis in POEMS syndrome-associated calciphylaxis.

Original languageEnglish
Pages (from-to)516-518
Number of pages3
JournalIndian Journal of Dermatology
Volume65
Issue number6
DOIs
Publication statusPublished - 2020 Nov 1

All Science Journal Classification (ASJC) codes

  • Dermatology

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