Department of Dermatology, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
J Dermatol. 2012 Nov;39(11):922-6. doi: 10.1111/j.1346-8138.2012.01609.x. Epub 2012 Jun 23.
We report a case of a 64-year-old man with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome that had been previously misdiagnosed as systemic sclerosis. He had typical symptoms of POEMS syndrome, however, the existence of skin sclerosis, contracture of fingers and pigmentation were similar to that of systemic sclerosis. Ten patients, including the patient discussed in this case, visited our department between 1990 and 2011. Among them, five patients had skin sclerosis. Therefore, we compared skin lesions and clinical/laboratory features of POEMS syndrome and systemic sclerosis in an attempt to distinguish these disorders. Regarding the cutaneous and laboratory findings, the existence of hemangioma or hypertrichosis is indicative of POEMS syndrome. By contrast, the existence of systemic sclerosis-specific autoantibodies, nail fold bleeding, digital ulcer/digital pitting scar or telangiectasia is highly suggestive of systemic sclerosis. To our knowledge, this is the first report to discuss in detail the differentiation between POEMS syndrome and systemic sclerosis.
我们报告了一例 64 岁男性患者，患有 POEMS（多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变）综合征，该患者先前被误诊为系统性硬化症。他具有典型的 POEMS 综合征症状，但皮肤硬化、手指挛缩和色素沉着与系统性硬化症相似。1990 年至 2011 年间，共有 10 名患者（包括本例患者）在我们科室就诊。其中 5 名患者有皮肤硬化。因此，我们比较了 POEMS 综合征和系统性硬化症的皮肤病变和临床/实验室特征，试图区分这些疾病。关于皮肤和实验室检查结果，血管瘤或多毛症的存在提示 POEMS 综合征。相比之下，存在系统性硬化症特异性自身抗体、甲襞出血、指溃疡/指压痕或毛细血管扩张高度提示系统性硬化症。据我们所知，这是首次详细讨论 POEMS 综合征和系统性硬化症的鉴别诊断。
