大疱性系统性红斑狼疮2例

Report of Two Cases with Bullous Lupus Erythematosus

报告2例大疱性系统性红斑狼疮,均为青年男性。以面部、躯干、四肢红斑、水疱为首发症状。例1患者白细胞总数1.85×109/L;24h尿蛋白定量1.040g/24h;ANA(++);抗SM(+);直接免疫荧光:IgG、IgA、IgM、C3基底膜荧光(+)。例2患者有踝关节疼痛;口腔溃疡;抗核抗体ANA(++);抗ds-DNA1/10阳性,抗SM(+);24h尿蛋白定量7.184g/24h。均符合系统性红斑狼疮诊断标准。两例患者皮肤组织病理结合直接免疫荧光符合大疱性系统性红斑狼疮病理改变。糖皮质激素联合氨苯砜等治疗,皮损全部消退。

To report two cases of patients with bullous lupus erythematosus（BLE）.Both of them were males at their early twenties,presented with blisters and bullae on the faces,trunks and extremities.The abnormalities of lab test in one case were showing with 1.85×109 of white blood test（WBC）,1.04g of total urine protein in 24hours,ANA＋＋,SM＋ and IgG,IgA,IgM and C3 deposition with direct immunofluorescence test.Besides the skin lesion,the other case also had arthralgia and oral ulceration.The lab test disorders included ANA＋＋,SM＋,dsDNA 1：10 and 7.184g of 24 hours urine protein count.The skin biopsies demonstrated the histological changes of BLE.Both patients had achieved improvement with administration of corticosteroids and depsone.