Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Disease (NCRC-DID), Ministry of Science & Technology; State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH); Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, 100730, China.
Orphanet J Rare Dis. 2022 Jul 23;17(1):290. doi: 10.1186/s13023-022-02445-z.
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.
大疱性系统性红斑狼疮（BSLE）是一种罕见的系统性红斑狼疮（SLE）亚型，临床上以表皮下紧张性水疱或大疱为特征。我们旨在探讨 BSLE 患者的临床和实验室特征。
我们回顾性分析了 2015 年至 2021 年在我院符合 BSLE 诊断标准的所有患者。评估了皮肤损害、全身表现、治疗选择和结局。对于每例 BSLE，从单一 SLE 患者中随机选择 4 例对照。比较两组之间的主要临床和实验室特征。
在 4221 例 SLE 患者中，有 12 例发生 BSLE。水疱性皮损是 5 例 BSLE 患者（5/12，41.7%）的首发症状，另外 7 例（7/12，58.3%）在 SLE 诊断后出现，从 SLE 发病到出现 BSLE 的中位时间为 36 个月（4-115 个月）。最常见的 BSLE 受累部位是头颈部（10/12，83.3%）、四肢（9/12，75.0%）、躯干（7/12，58.3%）和黏膜（6/12，50.0%）。所有 BSLE 患者均有皮肤外受累。10/12（83.3%）患者的 SLE 疾病活动指数评分超过 5，提示疾病活动度高。BSLE 组患者蛋白尿（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）和白细胞减少症（66.7% vs. 25.0%，P=0.006）的发生率明显高于对照组。全身皮质类固醇、免疫抑制剂、氨苯砜和皮肤护理的使用对控制疾病有效。
水疱性皮损可能是 BSLE 患者的首发表现，提示疾病活动度高。通过临床、组织病理学和免疫学评估进行早期诊断，可以对这种进行性疾病进行适当治疗，改善预后。
