LINEAR IGA BULLOUS DERMATOSIS OF CHILDHOOD WITH AUTOANTIBODIES TO A 230-KDA EPIDERMAL ANTIGEN

被引：30

作者：

KANITAKIS, J

MAUDUIT, G

COZZANI, E

BADINAND, P

FAURE, M

CLAUDY, A

机构：

[1] Department of Dermatology, Hôpital Ed. Herriot, Lyon

[2] Departments of Dermatology, Mâon

来源：

PEDIATRIC DERMATOLOGY | 1994年 / 11卷 / 02期

关键词：

D O I：

10.1111/j.1525-1470.1994.tb00568.x

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Linear IgA bullous dermatosis (LABD) is an autoimmune, subepidermal disease defined on the basis of direct immunofluorescence findings. However, more recent techniques used to study bullous dermatoses suggest that LABD may be heterogeneous. A patient with LABD of childhood (chronic benign disease of childhood, CBDC) was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen. This young girl's periorificial (mouth, genitalia), erythematovesicular lesions were diagnosed initially as herpes simplex. Histologic examination revealed eosinophilic spongiosis, suggesting the diagnosis of an autoimmune blistering disease. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermoepidermal junction. Indirect immunofluorescence revealed circulating IgA autoantibodies that reacted with the epidermal side of salt-split skin; these reacted by Western blot with a 230 kDa epidermal antigen, as in bullous pemphigoid. This case, fulfilling the diagnostic clinical and direct immunofluorescence criteria for LABD/CBDC, seems to represent IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.

引用

页码：139 / 144

页数：6

共 30 条

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