硬化性黏液水肿伴IgGκ型副蛋白血症一例  

Cleromyxedema with IgGκ paraproteinemia:a case report

作　　者：方梦 赵晴 曹珊 周桂芝[2] 张福仁[1,2] FANG Meng;ZHAO Qing;CAO Shan;ZHOU Guizhi;ZHANG Furen(Shandong University,Jinan 250012,China;Shandong Provincial Hospital for Skin Diseases&Shandong Provincial Institute of Dermatology and Venereology,Shandong First Medical University&Shandong Academy of Medical Sciences,Jinan 250022,China)

机构地区：[1]山东大学,济南250012 [2]山东第一医科大学附属皮肤病医院(山东省皮肤病医院),山东省皮肤病性病防治研究所,济南250022

出　　处：《中国麻风皮肤病杂志》2023年第4期277-279,共3页China Journal of Leprosy and Skin Diseases

基　　金：山东省皮肤性病学临床医学研究中心;山东第一医科大学学术提升计划(编号:2019LJ002)。

摘　　要：患者,男,67岁。全身丘疹伴硬化4年余。皮肤科查体:全身泛发粟粒大、蜡样坚实丘疹,部分融合成片,伴随皮肤发硬、弹性减退。皮损病理示:真皮层成纤维细胞增生、黏蛋白沉积。实验室检查:IgGκ型单克隆免疫球蛋白血症,ANA:1∶100弱阳性、IgA:0.88 g/L略降低。诊断:硬化性黏液水肿伴IgGκ型副蛋白血症。激素联合甲氨蝶呤治疗2个月后皮损明显好转。A 67-year-old male presented with papules and sclerosis all over the body for more than 4 years.Dermatological examination showed miliary-sized,waxy firm papules that partially coalesce into flakes,accompanied by firmness and loss of elasticity of the skin.Pathological showed that dermal fibroblast proliferation and mucin deposition.Laboratory examination showed IgG kappa monoclonal immunoglobulinemia,weakly positive ANA:1∶100,slightly lower IgA:0.88g/L.The diagnosis of scleromyxedema complicated with IgGκ paraproteinemia was made.The patient was treated with hormones and methotrexate for 2 months and the skin lesions improved significantly.

关 键 词：硬化性黏液水肿 IgGκ型单克隆免疫球蛋白 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]