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[疱疹样天疱疮]

[Pemphigus herpetiformis].

作者信息

Haensch R

出版信息

Hautarzt. 1979 Aug;30(8):418-22.

PMID:511546
Abstract

Pemphigus herpetiformis is a separate form of pemphigus with its own features. Clinically it resembles dermatitis herpetiformis Duhring. Periodically there is an extraordinary violent itching. Histologically pemphigus herpetiformis shows an eosinophilic spongiosis. The important acantholysis can only be found by repeated biopsies. Because of the acantholysis and the intercellular autoantibodies, demonstrated by direct and indirect immunfluorescence, this dermatosis belongs to the group of pemphigus. The therapeutical response to 4,4'-diaminodimethyl-diphenylsulfone (DDS)--a similarity to dermatitis herpetiformis Duhring--is especially characteristic. Our own observation gives occasion to characterize this pemphigus variant, which is repeatedly described in the literature under different designations, as pemphigus herpetiformis.

摘要

疱疹样天疱疮是天疱疮的一种独立形式,有其自身特点。临床上它类似杜林氏疱疹样皮炎。周期性地会出现异常剧烈的瘙痒。组织学上,疱疹样天疱疮表现为嗜酸性海绵形成。重要的棘层松解只能通过反复活检才能发现。由于棘层松解以及直接和间接免疫荧光显示的细胞间自身抗体,这种皮肤病属于天疱疮组。对4,4'-二氨基二甲基二苯砜(DDS)的治疗反应——与杜林氏疱疹样皮炎相似——尤为典型。我们自己的观察使我们有机会将这种在文献中以不同名称反复描述的天疱疮变体描述为疱疹样天疱疮。

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