POEMS症候群の皮膚症状

Skin Changes in POEMS Syndrome

POEMS症候群では浮腫，剛毛，色素沈着，血管腫，四肢末端チアノーゼなどの皮膚症状が高頻度に認められ，診断基準にも取り入れられている。皮膚症状のそれぞれは疾患特異的とは言えないが，複数が認められる場合には本症候群の可能性は非常に高まる。皮膚症状は本症候群において最も早期から発することから，その認識と理解は早期診断に大きく寄与する。本症候群に対する新規治療は2000年代から飛躍的に進歩しており，皮膚症状の正しい理解が早期発見・診断と予後の改善につながるものと思われる。

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.