AESOP综合征合并POEMS综合征1例国内首报  

A case of AESOP syndrome complicated by POEMS syndrome:the first case in China

作　　者：胡中慧 刘跃华[1] 王涛[1] Hu Zhonghui;Liu Yuehua;Wang Tao(Department of Dermatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院、北京协和医学院北京协和医院皮肤科,北京100730

出　　处：《中华皮肤科杂志》2025年第3期250-253,共4页Chinese Journal of Dermatology

基　　金：北京协和医院中央高水平医院临床科研专项;北京协和医院中央高水平医院临床科研专项。

摘　　要：患者女,58岁,因右侧肩部、胸部红斑8年就诊。红斑出现同期因双下肢麻木、无力,外周血M蛋白水平升高,甲状腺功能异常,右侧腋窝多发淋巴结肿大确诊POEMS综合征,行自体造血干细胞移植术后红斑消退,1年前红斑复发。体检:右侧颈部触及鸽蛋大小肿大淋巴结。皮肤科检查:右侧肩部、胸部片状红斑,边界较清,质硬。血清蛋白电泳示M蛋白升高,免疫固定电泳显示IgGλ限制性条带。外周血血管内皮生长因子水平升高。CT平扫示右侧肩胛骨肩峰端不规则片状骨质破坏区,符合骨浆细胞瘤表现。右肩部皮肤组织病理检查见真皮胶原间大量扩张增生的血管,诊断为AESOP综合征。给予来那度胺联合地塞米松方案化疗,治疗3周期后M蛋白转阴,红斑较前变暗,范围缩小。A 58-year-old female patient presented with erythema on the right shoulder and chest for 8 years.The appearance of erythema was concurrent with numbness and weakness in both lower limbs,elevated peripheral blood M protein levels,thyroid dysfunction,and multiple enlarged lymph nodes in the right axilla,leading to a diagnosis of POEMS syndrome.After autologous hematopoietic stem cell transplantation,erythema subsided but recurred 1 year ago prior to this visit.Physical examination revealed a pigeon egg-sized swollen lymph node on the right side of the neck.Skin examination showed patchy erythema on the right shoulder and chest,with clear boundaries and a hard texture.Serum protein electrophoresis revealed an elevated M protein level,and serum immunofixation electrophoresis showed a restricted IgGλband.In addition,peripheral blood levels of vascular endothelial growth factor were elevated.Computed tomography scan images showed an irregular flaky bone destruction area at the acromion end of the right scapula,which was consistent with the manifestations of bone plasmacytoma.Histopathological examination of the skin tissue in the right shoulder revealed a large number of dilated and proliferative vessels between dermal collagen fibers,and the patient was diagnosed with AESOP syndrome.Lenalidomide combined with dexamethasone chemotherapy was administered.After three cycles of treatment,M protein turned negative,and erythema became darker in color,and decreased in size compared with the skin lesion before treatment.

关 键 词：POEMS综合征 副肿瘤综合征 浆细胞瘤 AESOP综合征 血管内皮生长因子类 M蛋白 

分 类 号：R73[医药卫生—肿瘤]