Liu Cheng-Han, Chen Yu-Chen, Chen Tsai-Yun, Huang Hang-Wei, Lee Julia Yu-Yun
Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Division of Hematology and Oncology, Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Indian J Dermatol. 2020 Nov-Dec;65(6):516-518. doi: 10.4103/ijd.IJD_492_18.
POEMS syndrome, a paraneoplastic disorder, caused by plasma cell dyscrasia, is characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. Calciphylaxis is a vasculopathy of subcutaneous arteries with calcification and skin ulceration, most commonly associated with end-stage renal disease. Calciphylaxis occurring in patients with POEMS syndrome is very rare; only 8 cases have been reported to date. We report the 9 case. A middle-aged woman with chronic inflammatory demyelinating polyneuropathy and monoclonal plasma cell proliferative disorder with IgA gammopathy presented with a 3-month history of multiple painful violaceous plaques with gangrenous necrosis and ulceration on the abdomen and extremities. The skin lesions worsened after surgical debridement and fasciotomy but improved with weekly intravenous bortezomib combined with thalidomide and dexamethasone for POEMS syndrome. The present case illustrates that effective control of the disease activity of POEMS syndrome may improve the surgical outcome of extensive skin necrosis in POEMS syndrome-associated calciphylaxis.
POEMS综合征是一种由浆细胞异常增生引起的副肿瘤性疾病，其特征为多发性神经病、器官肿大、内分泌病、M蛋白和皮肤改变。钙化防御是一种皮下动脉血管病变，伴有钙化和皮肤溃疡，最常见于终末期肾病。POEMS综合征患者发生钙化防御非常罕见；迄今为止仅报道过8例。我们报告第9例。一名患有慢性炎症性脱髓鞘性多发性神经病和伴有IgA丙种球蛋白病的单克隆浆细胞增殖性疾病的中年女性，腹部和四肢出现多个疼痛性紫红色斑块，伴有坏疽性坏死和溃疡，病程3个月。手术清创和筋膜切开术后皮肤病变恶化，但每周静脉注射硼替佐米联合沙利度胺和地塞米松治疗POEMS综合征后病情改善。本病例表明，有效控制POEMS综合征的疾病活动可能改善POEMS综合征相关钙化防御中广泛皮肤坏死的手术结局。
