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儿童IgA线性皮肤病(儿童慢性大疱性疾病)。

IgA linear dermatosis of childhood (chronic bullous disease of childhood).

作者信息

Chorzelski T P, Jablonska S

出版信息

Br J Dermatol. 1979 Nov;101(5):535-42. doi: 10.1111/j.1365-2133.1979.tb11882.x.

Abstract

Of twenty-seven cases of subepidermal blistering disease of children twelve corresponded clinically, histologically and immunologically to dermatitis herpetiforms of adults, six to bullous pemphigoid, and eight to chronic bullous disease of childhood (CBDC), i.e. IgA linear dermatosis. This latter disease seems to be a distinct entity, different from both dermatitis herpetiformis and bullous pemphigoid, and is characterized immunopathologically by linear IgA deposits at the basement membrane zone. These cases usually do not show intestinal involvement and respond well to combined treatment with sulphones and corticosteroids, whereas sulphones or sulphapyridine alone are, even in very high doses, not sufficient for full control of the disease. CBDC or IgA linear dermatosis of childhood may be regarded as a counterpart of IgA linear dermatosis of adults.

摘要

在27例儿童表皮下疱病中,12例在临床、组织学和免疫学上与成人疱疹样皮炎相符,6例与大疱性类天疱疮相符,8例与儿童慢性大疱病(CBDC)即IgA线状皮病相符。后一种疾病似乎是一种独特的实体,与疱疹样皮炎和大疱性类天疱疮均不同,其免疫病理特征为基底膜带处有线性IgA沉积。这些病例通常不表现出肠道受累,对砜类药物和皮质类固醇联合治疗反应良好,而单独使用砜类药物或磺胺吡啶,即使剂量很高,也不足以完全控制疾病。儿童CBDC或IgA线状皮病可被视为成人IgA线状皮病的对应物。

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