Salgado Guerrero Maria, Mena Miranda Oscar, Arevalo Ana B, Barjaktarovic Nevena, Mendez Barbara
Department of Medicine, Jacobi Medical Center/Albert Einstein College of Medicine, Bronx, NY, USA.
Department of Medicine, Mount Sinai Morningside-West, New York, NY, USA.
Case Rep Rheumatol. 2020 Jul 25;2020:8873337. doi: 10.1155/2020/8873337. eCollection 2020.
Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with Systemic Lupus Erythematosus (SLE). BSLE can present as the initial manifestation of SLE and may be a marker of severe disease. In this case report, we present a case of a 22-year-old African American woman with BSLE and impaired renal function with subsequent nephrotic range proteinuria concerning for lupus nephritis and autoimmune hemolytic anemia, refractory to systemic corticosteroids, immunoglobulin, and mycophenolate mofetil, requiring dapsone after careful desensitization due to prior history of angioedema with sulfa drugs. This case highlights the importance of the prompt recognition of BSLE as the initial manifestations of SLE and illustrates the association of BSLE with severe disease and the benefit of concomitant use of dapsone with corticosteroids and other immunosuppressant drugs, even in patients with a history of sulfa allergy.
大疱性系统性红斑狼疮（BSLE）是一种罕见的皮肤自身免疫性疾病，其特征为系统性红斑狼疮（SLE）患者出现迅速、广泛的水疱大疱性皮损。BSLE可作为SLE的初始表现出现，且可能是严重疾病的一个标志。在本病例报告中，我们呈现了一名22岁非裔美国女性患BSLE且肾功能受损，随后出现肾病范围蛋白尿，考虑为狼疮性肾炎和自身免疫性溶血性贫血，对全身用糖皮质激素、免疫球蛋白及霉酚酸酯治疗无效，因既往有磺胺类药物所致血管性水肿病史，经仔细脱敏后需要使用氨苯砜。该病例强调了及时识别BSLE作为SLE初始表现的重要性，并说明了BSLE与严重疾病的关联以及氨苯砜与糖皮质激素和其他免疫抑制药物联合使用的益处，即使是有磺胺类药物过敏史的患者。
