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POEMS Syndrome with Extensive Calciphylaxis: A Case Report and Literature Review
Cheng-Han Liu1, Yu-Chen Chen1, Tsai-Yun Chen2
1Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Indian Journal of Dermatology
|January 25, 2021
Summary
POEMS syndrome-associated calciphylaxis is rare. Effective treatment of POEMS syndrome improved extensive skin necrosis, suggesting disease control enhances surgical outcomes.
Area of Science:
- Medicine
- Hematology
- Dermatology
Background:
- POEMS syndrome is a rare paraneoplastic disorder involving polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.
- Calciphylaxis is a severe vasculopathy causing painful skin ulcerations, typically linked to end-stage renal disease.
Observation:
- This report details the 9th known case of calciphylaxis in a patient with POEMS syndrome.
- The patient presented with painful, necrotic skin lesions that initially worsened after surgical intervention.
Findings:
- Treatment with bortezomib, thalidomide, and dexamethasone for POEMS syndrome led to improvement in the patient's extensive skin necrosis.
- This suggests a link between managing POEMS syndrome activity and the success of surgical interventions for associated calciphylaxis.
Implications:
- Effective management of POEMS syndrome may be crucial for improving surgical outcomes in patients with calciphylaxis.
- This case highlights the importance of considering POEMS syndrome in patients with calciphylaxis and underscores the potential benefit of systemic therapy.
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