Department of Dermatology, Program in Epithelial Biology, Stanford University School of Medicine, CA 94305, USA.
Clin Dermatol. 2012 Jan-Feb;30(1):38-50. doi: 10.1016/j.clindermatol.2011.03.008.
Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time.
线性免疫球蛋白 A(IgA)大疱性皮病,又称线性 IgA 病,是一种自身免疫性黏膜皮肤疾病,其特征为表皮下大疱,IgA 自身抗体针对基底膜带中的几种不同抗原。其免疫病理学特征在于基底膜带上存在连续的线性 IgA 沉积,直接免疫荧光下清晰可见。与儿童发病的线性 IgA 病相比,成人发病的线性 IgA 病具有不同的临床特征和分布。通过临床、组织病理学和免疫病理学检查来进行诊断。两种常见的治疗方法是氨苯砜和磺胺吡啶,它们可以减轻炎症反应,并在不同的时间内实现疾病缓解。