硬化性黏液水肿合并嗜酸性粒细胞增多症一例并文献复习  

Scleromyxedema associated with eosinophilia: a case report and literature review

作　　者：何春霞[1] 王涛[1] 王诗琪[1] 渠涛[1] HE Chunxia;WANG Tao;WANG Shiqi;QU Tao(Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & 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

出　　处：《中国麻风皮肤病杂志》2019年第7期422-424,共3页China Journal of Leprosy and Skin Diseases

基　　金：中国医学科学院医学与健康科技创新工程服务“一带一路”战略先导科研专项(编号:2017-12M-B&R-01)

摘　　要：报告1例硬化性黏液水肿合并嗜酸性粒细胞增多症并对相关文献进行复习。患者,男,37岁。关节疼痛14个月,皮疹10个月。皮疹表现为全身泛发、粟粒大小、蜡样坚实丘疹。皮肤组织病理提示真皮成纤维细胞增生、黏蛋白沉积。实验室检查提示IgGλ型单克隆免疫球蛋白血症、外周血嗜酸粒细胞计数显著升高。经静脉输注人免疫球蛋白(IVIG)联合激素、沙利度胺等治疗,患者皮疹及关节症状显著缓解。A case of scleromyxedema associated with eosinophilia is reported and relevant literature is reviewed. A 37-year-old male presented with arthralgia for 14 months and generalized waxy papules for 10 months. Histopathology revealed mucin deposition and fibroblast proliferation in the dermis. Laboratory tests demonstrated the high level of monoclonal gammopathy of IgGλ and peripheral eosinophilia. The patient achieved a dramatic improvement of the skin lesions and arthralgia after IVIG treatment combined with systemic corticosteroid and thalidomide for 6 months.

关 键 词：硬化性黏液水肿 嗜酸性粒细胞增多症 人免疫球蛋白 

分 类 号：R758.4[医药卫生—皮肤病学与性病学]