[患有针对180 kDa类天疱疮抗原自身抗体的儿童线状IgA大疱性皮肤病]

[Linear IgA bullous dermatosis in children with autoantibodies against 180 kDa pemphigoid antigen].

作者信息

Bérard F, Kanitakis J, Di Maio M, Ghohestani R, Hermier C, David L, Claudy A, Faure M

机构信息

Clinique dermatologique, hôpital Edouard-Herriot, Lyon, France.

出版信息

Arch Pediatr. 1996 Apr;3(4):345-7. doi: 10.1016/0929-693x(96)84689-6.

DOI:10.1016/0929-693x(96)84689-6

PMID:8762956

Abstract

BACKGROUND

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease defined on the basis of direct immunofluorescence findings.

CASE REPORT

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.

CONCLUSION

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.

摘要

背景

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。

病例报告

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。