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报告1例Waldenstrom高γ球蛋白血症型紫癜(简称HGP)并发干燥综合征。患者女,40岁。反复出现双下肢瘀点、瘀斑6年,长时间站立或行走后诱发或者加重。皮肤科检查:双下肢以小腿为主密布暗红色瘀点、瘀斑,伴褐色色素沉着。外周血示高γ球蛋白,真皮浅层血管壁有大量IgG沉积。根据临床表现、实验室检查及免疫荧光检查结果诊断为HGP。患者伴有口干、眼干,唾液腺分泌功能降低,Schirmer I试验阳性,提示并发干燥综合征。使用雷公藤多苷60 mg/d联合秋水仙碱1 mg/d口服,一周后皮疹得到有效控制。
One case reported Waldenstrom hypergammaglobulinemia Purpura (referred to as HGP) complicated with Sjogren’s syndrome. Female patient, 40 years old. Repeated lower extremity petechia, ecchymosis 6 years, a long time standing or walking induced or aggravated. Dermatology examination: double lower extremity to the main dark clouds petechia, ecchymosis, with brown pigmentation. Peripheral blood showed high gamma globulin, a large number of deposition of IgG on the superficial dermal wall. According to clinical manifestations, laboratory tests and immunofluorescence results were diagnosed as HGP. Patients with dry mouth, dry eyes, salivary gland secretion decreased, Schirmer I test positive, suggesting concurrent Sjogren’s syndrome. Tripterygium glycosides 60 mg / d combined with colchicine 1 mg / d orally, a week after the rash was effectively controlled.