大疱性系统性红斑狼疮五例分析

目的 探讨大疱性系统性红斑狼疮的临床及病理特征,提高对该病的认识.方法 对5例大疱性系统性红斑狼疮患者皮损进行组织病理检查,并用鼠抗人免疫球蛋白(IgG,IgM,IgA)及补体C3进行直接免疫荧光检查,对临床、组织病理特点及治疗预后情况进行分析.结果 皮损好发于面部、上肢等曝光部位,临床表现为正常皮肤上出现水疱或者大疱,5例患者病理的典型改变为表皮下水疱,水疱内及真皮乳头可见较多中性粒细胞浸润.5例患者免疫病理均显示免疫球蛋白和(或)补体在基底膜带呈带状沉积.患者对氨苯砜治疗反应较好,糖皮质激素联合免疫抑制有效.结论 大疱性系统性红斑狼疮可能与狼疮肾炎活动有关.氨苯砜联合糖皮质激素或者糖皮质激素联合免疫抑制剂治疗有效.

Clinicopathological analysis of five cases of bullous systemic lupus erythematosus

Objective To investigate the clinical and histopathological features of bullous systemic lupus erythematosus (BSLE),and to improve understanding of BSLE.Methods Lesions from 5 cases of BSLE were subjected to histopathological examination,and direct immunofluorescence (DIF) assay with mouse antibodies against human immunoglobulins (IgG,IgM,IgA) and complement component C3.The clinical and histopathological features,treatment and prognosis of BSLE were retrospectively analyzed.Results Skin lesions mostly occurred in sun-exposed areas such as the face and upper extremities,and included blisters or bullae arising on normal-appearing skin.Typical histopathological changes in these patients were subepidermal blisters,and infiltration of plenty of neutrophils within blisters and dermal papillae.Immunopathological study showed band-like deposition of immunoglobulins and/or complement along the basement membrane zone.Patients with BSLE responded well to dapsone,and glucocorticoids combined with immunosuppressive agents were also effective for the treatment of BSLE.Conclusions BSLE may be related to lupus nephritis activity.Glucocorticoids combined with dapsone or with immunosuppressive agents are effective for the treatment of BSLE.