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疱疹样大疱性表皮松解症(Dowling-Meara型)。1例报告及病理形态发生学研究

Epidermolysis bullosa herpetiformis Dowling-Meara. Report of a case and pathomorphogenesis.

作者信息

Anton-Lamprecht I, Schnyder U W

出版信息

Dermatologica. 1982 Apr;164(4):221-35.

PMID:7084543
Abstract

Report of a 2-year-old girl of Turkish origin with congenital generalized blister formation in herpetiform arrangement. Direct immunofluorescence ruled out juvenile dermatitis herpetiformis Duhring. Ultrastructural investigation of a fresh blister and clinically intact preblistering skin revealed intraepidermal blister formation via cytolysis of basal cells preceded by clumping of tonofilaments and partial attachment to the hemidesmosomes at the dermo-epidermal junction. This type of blister formation is significantly different from all other epidermolysis bullosa types and is a characteristic feature of all further cases studied so far by electron microscopy (n = 20) which correspond to the original cases of Dowling and Meara. Clinical features characteristic of this epidermolysis type are outlined and classification of epidermolysis bullosa herpetiformis Dowling-Meara into the non-scarring epidermolyses with dominant inheritance is discussed.

摘要

一名2岁土耳其裔女孩的报告,其先天性全身性水疱呈疱疹样排列。直接免疫荧光排除了杜林型青少年疱疹样皮炎。对一个新鲜水疱和临床上完整的水疱前期皮肤进行超微结构研究发现,表皮内水疱形成是通过基底细胞溶解,之前有张力丝聚集以及在真皮 - 表皮交界处部分附着于半桥粒。这种水疱形成类型与所有其他大疱性表皮松解症类型显著不同,并且是迄今为止通过电子显微镜研究的所有进一步病例(n = 20)的特征性表现,这些病例与道林和米拉的原始病例相对应。概述了这种表皮松解症类型的临床特征,并讨论了将道林 - 米拉型疱疹样大疱性表皮松解症归类为具有显性遗传的非瘢痕性表皮松解症。

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Epidermolysis bullosa herpetiformis Dowling-Meara. Report of a case and pathomorphogenesis.疱疹样大疱性表皮松解症(Dowling-Meara型)。1例报告及病理形态发生学研究
Dermatologica. 1982 Apr;164(4):221-35.
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[Dowling-Meara dominant epidermolysis bullosa. An intraepidermal epidermolysis bullosa which hides its prognosis well].[Dowling-Meara显性大疱性表皮松解症。一种预后隐匿的表皮内大疱性表皮松解症]
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[Ultrastructure of epidermolyses with junctional blister formation (author's transl)].
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[Dowling-Meara Epidermolysis bullosa herpetiformis].[Dowling-Meara型疱疹样大疱性表皮松解症]
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Keratin expression in epidermolysis bullosa simplex (Dowling-Meara).单纯性大疱性表皮松解症(Dowling-Meara型)中的角蛋白表达。
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Epidermolysis bullosa nevus arising in a patient with Dowling-Meara type epidermolysis bullosa simplex with a novel K5 mutation.一名患有Dowling-Meara型单纯性大疱性表皮松解症且存在新型K5突变的患者发生了大疱性表皮松解痣。
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Epidermolysis bullosa herpetiformis Dowling-Meara in a large family.一个大家族中的疱疹样大疱性表皮松解症(Dowling-Meara型)
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