[Skin Changes in POEMS Syndrome].

Brain and nerve = Shinkei kenkyu no shinpo 2019

["Kuwabara S"]

Abstract

Skin changes, including peripheral edema, hyperpigmentation, hypertrichosis, and hemangioma are frequent and early manifestations of POEMS (polyneuropathy, organomegaly, enndocrinopathy, M-protein, and skin changes) syndrome. Hemangiomata is presumably caused by overproduction of the vascular endothelial growth factor, and hypertrichosis results from the deposition of melanin; however, the pathogenesis of the skin symptoms are poorly understood. Nevertheless, recognition of the skin changes facilitates early diagnosis and treatment. Novel treatments, such as autoplantation, thalidomide/lenalidomide, and proteasome inhibitors have been availoble since 2000, and outcomes of the disorder have significantly improved. Appropriate understanding of the skin manifestations is clinically important for the early diagnosis of this intractable disorder.

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