Department of Rheumatology, Ochsner Medical Center, New Orleans, LA, USA.
Department of Dermatology, Mayo Clinic Health System, Eau Claire, WI, USA.
Mod Rheumatol Case Rep. 2023 Jan 3;7(1):52-56. doi: 10.1093/mrcr/rxac046.
Cutaneous lupus erythematosus (CLE) is one of the most common manifestations of systemic lupus erythematosus (SLE), although it can manifest as an independent entity as well. Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous manifestation of SLE presenting as tense vesiculobullous eruptions in a photosensitive distribution. Pathophysiology is secondary to autoantibodies against noncollagenous domain 1 and 2 (NC1 and NC2) type VII collagen, and histopathology reveals dense neutrophilic infiltration of the dermis with direct immunofluorescence showing IgG deposition at dermoepidermal junction. There is lack of data on available therapeutic options to treat BSLE, and varying responses to dapsone, methotrexate, azathioprine and corticosteroids have been reported. Belimumab, a fully humanised Change to Immunoglobulin G1λ (IgG1λ) monoclonal antibody targeting soluble B lymphocyte stimulator protein, was the first Food and Drug Administration-approved drug for SLE and has been reported to be effective for CLE. We present the case of a 41-year-old black female with SLE presenting with BSLE, who was successfully treated with corticosteroids and belimumab and did not experience disease relapse even after discontinuation of corticosteroids. To our knowledge, this is the first reported case of successful treatment of BSLE with belimumab, and further research can help determine the role of belimumab in the treatment of BSLE.
皮肤红斑狼疮（CLE）是系统性红斑狼疮（SLE）最常见的表现之一，尽管它也可以作为一种独立的实体表现出来。大疱性系统性红斑狼疮（BSLE）是 SLE 的一种罕见皮肤表现，表现为光敏分布的紧张性水疱性皮疹。发病机制继发于针对非胶原域 1 和 2（NC1 和 NC2）VII 型胶原的自身抗体，组织病理学显示真皮致密中性粒细胞浸润，直接免疫荧光显示 IgG 在表皮真皮交界处沉积。目前尚无关于治疗 BSLE 的有效治疗方法的数据，据报道，达普司他、甲氨蝶呤、硫唑嘌呤和皮质类固醇的反应各不相同。贝利木单抗是一种针对可溶性 B 淋巴细胞刺激蛋白的全人源化 IgG1λ 单克隆抗体，是第一个获得美国食品和药物管理局批准用于治疗 SLE 的药物，据报道对 CLE 有效。我们报告了一例 41 岁的黑人女性患有 SLE，表现为 BSLE，她成功地接受了皮质类固醇和贝利木单抗治疗，即使在停用皮质类固醇后也没有出现疾病复发。据我们所知，这是首例成功用贝利木单抗治疗 BSLE 的病例报告，进一步的研究可以帮助确定贝利木单抗在治疗 BSLE 中的作用。
