大疱性系统性红斑狼疮文献回顾性分析

Retrospective analysis of bullous systemic lupus erythematousus

目的探讨大疱性系统性红斑狼疮临床、诊断及治疗特征,提高临床诊治水平。方法采用回顾性研究方法,对2001年至2019年中国期刊全文数据库报道的44例大疱性系统性红斑狼疮的临床特点、实验室检查、皮肤病理特征、治疗及预后进行分析。结果 44例大疱性系统性红斑狼疮患者平均年龄(27±12)岁,男女比例为1∶5.2。其中27.3%患者既往有SLE病史,15.9%被误诊为其他大疱性疾病,43.2%全身可见红斑、水疱,20.5%累及口腔黏膜。44例患者ANA均为阳性,病检均表现为表皮下水疱,72.7%出现真皮中性粒细胞浸润;75%患者肾脏受累,79.5%血液系统受累,95.5%使用糖皮质激素治疗。结论大疱性系统性红斑狼疮病情重、误诊率高。临床医生应加强与其他大疱性疾病的鉴别,积极评估患者系统受累情况,及早选择适宜的治疗方案以控制病情进展。

Objective To investigate the clinical, diagnostic and therapeutic characteristics of bullous systemic lupus erythematosus(BSLE), so as to improve the level of clinical diagnosis and treatment. Methods A retrospective analysis of clinical features, laboratory examination, dermatopathology, treatment and prognosis of 44 BSLE cases was conducted during the year from 2001 to 2019 via Chinese journal database. Results The average age of 44 patients is(27+12) years old, and the male to female ratio is 1:5.2. 27.3% of the patients had a history of SLE, 15.9% were misdiagnosed as other bullous disease, 43.2% presented erythema and blisters all over the body, 20.5% had mucous membrane involvement. All of the patients showed sub-epidermal blister in histological examination, and 72.7% of them with dermal infiltration of neutrophils. ANA are positive in all patients. 75% of the patients had renal involvement, 79.5% had hematological system involvement. 95.5% of the patient were improved by using glucocorticoid. Conclusion BSLE is a severe disease with high misdiagnosis rate. Strengthen the differential diagnosis with other bullous diseases, assess the systemic involvement actively and select the appropriate treatment to control the progress of the disease in the early time.