Bérard F, Kanitakis J, Di Maio M, Ghohestani R, Hermier C, David L, Claudy A, Faure M
Clinique dermatologique, hôpital Edouard-Herriot, Lyon, France.
Arch Pediatr. 1996 Apr;3(4):345-7. doi: 10.1016/0929-693x(96)84689-6.
Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease defined on the basis of direct immunofluorescence findings.
An 18 month-old girl suffering from LABD was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermal-epidermal junction. Indirect immunofluorescence revealed circulating autoantibodies that reacted with the epidermal side of salt-split skin; they reacted by Western blot with a 180 kDa epidermal antigen, as in bullous pemphigoid.
This dermatosis fulfilling the clinical features and direct immunofluorescence criteria for childhood LABD seems to represent a case of IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.
线状 IgA 大疱性皮肤病（LABD）是一种基于直接免疫荧光检查结果定义的自身免疫性表皮下大疱性疾病。
对一名患有 LABD 的 18 个月大女孩进行了盐裂皮肤间接免疫荧光检查和免疫印迹分析，以试图鉴定所涉及的自身抗原。直接免疫荧光显示在真皮 - 表皮交界处有唯一的线状 IgA 沉积。间接免疫荧光显示循环自身抗体与盐裂皮肤的表皮侧发生反应；它们在免疫印迹中与一种 180 kDa 的表皮抗原发生反应，如同在大疱性类天疱疮中一样。
这种符合儿童 LABD 临床特征和直接免疫荧光标准的皮肤病似乎代表了一例 IgA 大疱性类天疱疮。这进一步强调了 LABD 的疾病分类异质性，除了大疱性类天疱疮外，可能还包括获得性大疱性表皮松解症和瘢痕性类天疱疮。
