Waldenstrm高γ球蛋白血症型紫癜并发干燥综合征

Hypergammaglobulinemic purpura of Waldenstrm complicated with Sjogren's syndrome

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。

We report a case of hypergammaglobulinemic purpura of Waldenstrom （HGP） complicated with Sjogren＇s syn- drome. A 40-year-old woman presented with a 6-year history of recurrent leg petechiae. The aggravating factors included prolonged standing and walking. Physical examination revealed petechiae and brown pigmentation on both legs. The patient showed hypergammaglobulinemia, and immunofluorescence studies showed positive for IgG on the vessel wall. The patient had dry mouth/eyes, and the salivary gland＇s function was compromised. The skin lesions improved one-week after combined therapy of Tripterygium glucosides and colchicine.