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Cutaneous Manifestations in POEMS Syndrome: Case Report and Review
Flauberto Sousa Marinho1, Rodrigo Pirmez1, Renata Nogueira2
1Sector of Dermatology and Post-Graduation Course, University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
Abstract:
The authors report a case of sensorimotor polyneuropathy, diffuse cutaneous hyperpigmentation, skin sclerodermiform thickening and papular lesions in the infraclavicular and abdominal region. Besides weight loss, there were diabetes mellitus and hypothyroidism. The alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Cutaneous alterations are present in 68% of patients with diffuse cutaneous hyperpigmentation, plethora and acrocyanosis. Leukonychia, necrotizing vasculitis, hypertrichosis and cutaneous thickening of sclerodermiform type are also cited. The onset of multiple cutaneous angiomas in this syndrome has been observed in 24-44% of patients.
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