Department of Dermatology, Wuhan No. 1 Hospital, Wuhan, China.
Front Immunol. 2024 Aug 5;15:1409556. doi: 10.3389/fimmu.2024.1409556. eCollection 2024.
Linear IgA bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering disorder. Diagnosis always relies on skin pathology and direct immunofluorescence (DIF), with typical linear deposits of IgA along the basement membrane zone (BMZ). The typical clinical manifestation is tense bullae arranged like the "string of pearls" companied with severe pruritus. Dapsone is often considered first-line therapy for LABD, and it is necessary to test the HLA-B1301 gene to prevent the occurrence of dapsone-induced hypersensitivity syndrome (DHS). Here we report a case of LABD resistant to corticosteroid and sulfasalazine, while waiting for HLA-B1301 gene test results, dupilumab was used to control severe pruritus.
线性 IgA 大疱性皮病(LABD)是一种获得性自身免疫性表皮下水疱性疾病。诊断始终依赖于皮肤病理学和直接免疫荧光(DIF),可见 IgA 沿基底膜带(BMZ)呈典型线性沉积。典型临床表现为紧张性水疱排列如“珍珠串”,伴有严重瘙痒。氨苯砜通常被认为是 LABD 的一线治疗药物,有必要检测 HLA-B1301 基因以预防氨苯砜诱导的过敏反应综合征(DHS)的发生。在这里,我们报告了一例对皮质类固醇和柳氮磺胺吡啶耐药的 LABD 病例,在等待 HLA-B1301 基因检测结果的同时,使用度普利尤单抗来控制严重瘙痒。