Lee Mi Ra, Choi Hyo Jin, Lee Eun Bong, Baek Han Joo
Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, 1198 Kuwol-dong, Namdong-gu, Incheon, 405-760, South Korea.
Clin Rheumatol. 2007 Nov;26(11):1989-92. doi: 10.1007/s10067-007-0607-0. Epub 2007 Apr 5.
POEMS syndrome--characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes--is a rare multi-systemic disease. Its skin change is often like that of connective tissue diseases such as scleroderma. Although clinical manifestations of POEMS syndrome are known to be diverse, arterial or venous thrombosis is a less-recognised feature. We report a 41-year-old man with scleroderma-like skin change who was initially negative for monoclonal protein, but finally confirmed as having POEMS syndrome. During the disease course, he was complicated by extensive arterial thromboses including stroke. This case evokes the need to include POEMS syndrome in the differential diagnoses in patients with scleroderma-like skin change and to repeat measurements of monoclonal protein to confirm its diagnosis. A POEMS patient with extensive arterial thromboses may have a catastrophic disease course, requiring earlier diagnosis and more aggressive treatment.
POEMS综合征——其特征为多发性神经病、脏器肿大、内分泌病、单克隆蛋白血症和皮肤改变——是一种罕见的多系统疾病。其皮肤改变常类似于硬皮病等结缔组织病。尽管已知POEMS综合征的临床表现多样，但动脉或静脉血栓形成是一种较少被认识到的特征。我们报告一名41岁男性，有类似硬皮病的皮肤改变，最初单克隆蛋白检测为阴性，但最终确诊为POEMS综合征。在病程中，他并发了包括中风在内的广泛动脉血栓形成。该病例提示，对于有类似硬皮病皮肤改变的患者，鉴别诊断时需要考虑POEMS综合征，并重复检测单克隆蛋白以确诊。一名患有广泛动脉血栓形成的POEMS患者可能有灾难性的病程，需要更早诊断和更积极的治疗。
