与意义未明的单克隆球蛋白血症相关的白细胞碎裂性血管炎1例  

Leukocytoclastic Vasculitis Associated with Monoclonal Globulinemia of Undetermined Sig-nificance:A Case Report

作　　者：冯曦微 李桐[1] 王婷婷[1] FENG Xiwei;LI Tong;WANG Tingting(Department of Dermatovenereology,West China Hospital Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院皮肤性病科,四川成都610041

出　　处：《中国皮肤性病学杂志》2023年第4期470-472,共3页The Chinese Journal of Dermatovenereology

摘　　要：患者女,45岁,臀部皮疹反复发作伴轻度疼痛3年,并渐累及躯干、四肢、手足和面部。皮损组织病理示:白细胞碎裂性血管炎改变;直接免疫荧光示:真皮全层小血管壁IgA沉积。免疫球蛋白定量IgA 3380 mg/L(836~2900 mg/L);血清蛋白电泳查见异常M带,免疫固定电泳检测出单克隆IgAλ型条带。骨髓活检提示有8%克隆性浆细胞。诊断:(1)白细胞碎裂性血管炎;(2)意义未明的单克隆球蛋白血症。治疗:予以糖皮质激素、柳氮磺吡啶等治疗,皮损大部分消退。A 45-year-old female presented with recurrent annular erythema and plaques on the buttocks and over the extensor surface of four limbs for 3 years.Her trunk was affected gradually.A biopsy specimen showed the characteristic changes of leukocytoclastic vasculitis.Direct immunofluorescence showed IgA deposits in the blood vessels of whole dermis.Abnormal laboratory findings were elevated serum IgA level of 3380 mg/L(836-2900 mg/L),serum M protein level of 4%and monoclonal IgA lambda-type.The bone marrow aspiration demonstrated 8%monoclonal plasma cells.The diagnosis of leukocytoclastic vasculitis and monoclonal gammopathy of undetermined significance was made.Treatment with glucocorticoid and sulfasalazine was given,and the lesions were resolved.

关 键 词：单克隆球蛋白血症 白细胞碎裂性血管炎 环状皮损 

分 类 号：R593.2[医药卫生—内科学] R751[医药卫生—临床医学]