大疱性系统性红斑狼疮（BSLE）是系统性红斑狼疮（SLE）的一种罕见亚型，其临床特征是表皮下紧张的囊泡或大疱性。我们旨在探讨BSLE患者的临床和实验室特征。
我们回顾性回顾了2015年至2021年在我们机构中符合BSLE诊断标准的所有患者。皮肤损伤,系统性表现，治疗方案,并对结果进行了评估。对于BSLE的每种情况，从单发SLE患者中随机选择4名对照.比较两组患者的主要临床及实验室特征。
在4221例SLE患者中，12开发的BSLE。BSLE患者中有5例（5/12，41.7％）是首次出现，其余7例（7/12，58.3％）在SLE诊断后出现，从SLE开始的中位持续时间为36个月（4-115个月）。最常见的BSLE感染部位是头颈部（10/12，83.3%），四肢（9/12，75.0%），树干(7/12，58.3%)，粘膜（6/12，50.0%）。所有BSLE患者均有皮肤外受累。10/12(83.3%)患者SLE疾病活动指数得分超过5分,这表明疾病活动性高。BSLE组患者的蛋白尿发生率明显较高（83.3％vs.47.9%,P=0.027),血尿（75%vs.31.3%,P=0.006),溶血性贫血（33.3%vs.0%,P=0.000),和白细胞减少(66.7%vs.25.0%,P=0.006）比对照组高。全身性皮质类固醇的使用，免疫抑制剂,氨苯砜,皮肤护理在控制疾病方面是有效的。
囊泡状病变可能是BSLE患者的第一表现，表明疾病活动性高。早期诊断使用临床，组织病理学,和免疫学评估可以导致这种进行性疾病的适当治疗并改善预后。
Bullous systemic lupus erythematosus (BSLE) is a rare subtype of systemic lupus erythematosus (SLE) that is clinically characterized by subepidermal tense vesicles or bullae. We aimed to investigate the clinical and laboratory features of patients with BSLE.
We retrospectively reviewed all patients who fulfilled the diagnostic criteria for BSLE in our institution from 2015 to 2021. Cutaneous lesions, systemic manifestations, treatment options, and outcomes were evaluated. For each case of BSLE, four controls were randomly selected from patients with single SLE. Major clinical and laboratory characteristics were compared between the two groups.
Among 4221 patients with SLE, 12 developed BSLE.
Vesiculobullous lesions were the first sign in five of the BSLE patients (5/12, 41.7%) and appeared after SLE diagnosis in the remaining seven patients (7/12, 58.3%), with a median duration from SLE onset of 36 months (4-115 months). The most common BSLE-affected sites were the head and neck (10/12, 83.3%), extremities (9/12, 75.0%), trunk (7/12, 58.3%), and mucosae (6/12, 50.0%). All patients with BSLE had extra-cutaneous involvement. The SLE disease activity index score exceeded 5 in 10/12 (83.3%) patients, which indicated high disease activity. Patients in the BSLE group had significantly higher incidences of proteinuria (83.3% vs. 47.9%, P = 0.027), hematuria (75% vs. 31.3%, P = 0.006), hemolytic anemia (33.3% vs. 0%, P = 0.000), and leukopenia (66.7% vs. 25.0%, P = 0.006) than those in the control group. The use of systemic corticosteroids, immunosuppressants, dapsone, and skin care was effective in controlling disease.
Vesiculobullous lesions may be the first manifestation and indicate a high disease activity in patients with BSLE. Early diagnosis using clinical, histopathological, and immunological evaluations can lead to appropriate treatment of this progressive disease and improve prognosis.
