以寒冷性荨麻疹为表现的混合型冷球蛋白血症  

A case of mixed cryoglobulinemia presented with cold urticaria

作　　者：王岚琦[1] 王孝盼 赵忻 施若非[1] 赵肖庆[1] 郑捷[1] WANG Lan-qi;WANG Xiao-pan;ZHAO Xin;SHI Ruo-fei;ZHAO Xiao-qing;ZHENG Jie(Department of Dermatology,Ruijin Hospital,School of Medicine,Shanghai Jiaatong University,Shanghai 200025,Chia)

机构地区：[1]上海交通大学医学院附属瑞金医院皮肤科,上海200025

出　　处：《临床皮肤科杂志》2018年第9期574-577,共4页Journal of Clinical Dermatology

基　　金：国家自然科学基金青年(81502718)资助项目

摘　　要：报告1例以寒冷性荨麻疹为表现的混合性冷球蛋白血症。患者女,59岁。遇冷后面部及四肢出现瘙痒性风团5年。风团24 h内可消退,退后不留痕迹。病程中全身多关节疼痛。实验室检查:冷球蛋白定性试验阳性,血M蛋白阳性,为IgM-κ链型。皮损组织病理显示真皮浅层血管壁纤维蛋白样坏死,结构破坏,中性粒细胞浸润伴核尘,符合白细胞碎裂性血管炎。诊断:混合型冷球蛋白血症。予沙利度胺和白芍总苷治疗,患者病情好转。A case of mixed cryoglobulinemia presented with cold urticaria is reported. A 59-year-old Chinese woman complained of multiple joint pain, and recurrent and pruritic wheal on the cold-exposure sites, which resolved within 24 hours after getting warm, without hyperpigmentation, for 5 years. Laboratory tests revealed positive for both cIToglobulin and M protein（monoclonal IgM-K）. Histopathology showed fibrinoid necrosis of the damaged vessel walls in the upper dennis, transmural iniltration of the walls of small vessels by neutrophils, degranulation and fragmentation of neutrophils, leading to the production of nuclear dust. The histopathologieal features are refen＇ed to leukoeytoclastic vaseulitis. The diagnosis of mixed cryoglobulinemia was made. Clinical signs and symptoms were improved following the treatments with thalidomide and total glucoside of paeony capsules.

关 键 词：寒冷胜荨麻疹 冷球蛋白血症 沙利度胺 

分 类 号：R553[医药卫生—血液循环系统疾病]