POEMS Syndrome with Extensive Calciphylaxis: A Case Report and Literature 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 9th case. 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.

journal_name

Indian J Dermatol

authors

Liu CH,Chen YC,Chen TY,Huang HW,Lee JY

doi

10.4103/ijd.IJD_492_18

subject

Has Abstract

pub_date

2020-11-01 00:00:00

pages

516-518

issue

6

eissn

0019-5154

issn

1998-3611

pii

IJD-65-516

journal_volume

65

pub_type

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