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register hereLinear 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.
在此登记线性IgA大疱性皮肤病(LABD)是一种获得性自身免疫性表皮下起泡性疾病。诊断总是依赖于皮肤病理学和直接免疫荧光(DIF),沿基底膜区(BMZ)有典型的线性IgA沉积。典型的临床表现是紧张的大疱,排列成“珍珠串”,伴有严重的瘙痒。
Dapsone is often considered first-line therapy for LABD, and it is necessary to test the HLA-B*1301 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-B*1301 gene test results, dupilumab was used to control severe pruritus..
氨苯砜通常被认为是LABD的一线治疗方法,有必要测试HLA-B*1301基因以预防氨苯砜诱导的超敏反应综合征(DHS)的发生。在这里,我们报告了一例对皮质类固醇和柳氮磺吡啶耐药的LABD病例,在等待HLA-B*1301基因检测结果时,dupilumab被用于控制严重瘙痒症。。