Epidermolysis bullosa simplex (intraepidermal epidermolysis bullosa) and allied conditions
Epidermolysis bullosa simplex (intraepidermal epidermolysis bullosa) and allied conditions
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单纯性大疱性表皮松解症（表皮内大疱性表皮松解症）及相关病症
DOI：
10.1007/978-1-4899-7190-6_14
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发表时间：
1990
影响因子：
3.5
通讯作者：
T. Gedde
中科院分区：
文献类型：
--
作者：
T. Gedde
在孟德尔遗传定律被重新发现的30年前，von Hebra将表皮内无瘢痕性水疱称为Erblichen天疱疮[1]。Kobner在1886年将相同的病症称为遗传性大疱性表皮病（EBH）[2]。由于后一术语很快被用于几种先天性创伤性起泡疾病，Hallopeau [3]发现有必要将原始类型称为“简单”，即“简单”。e.单纯大疱性表皮炎（EBS）。到1908年，EBS被认为是显性孟德尔性状[4]。顺便说一句，Kobner的病例主要是手和脚的水泡，其他地方只有偶尔的水泡。许多类似的家族后来被称为局限性EBS（Weber-Cockayne），以Weber（1926）的孤立性“足部复发性大疱性出疹”病例[5]和Cockayne（1938）的两个仅足部起泡的优势家族[6]命名。这种情况以前被称为EBS [7]，但Cockayne将它们与EBS区分开来。自1957年以来，EBS（Weber-Cockayne）已用于脚或手和脚起泡的情况，EBS（Kobner）用于更广泛的起泡。Cockayne的一个家族具有“容易擦伤皮肤”的额外特征，因此可能代表1970年首次区分的不同疾病（EBS Ogna）[9]。
Thirty years before the rediscovery of Mendel’s laws of inheritance intraepidermal non-scarring blistering was called Erblichen Pemphigus by von Hebra [1]. Kobner in 1886 called the same condition epidermolysis bullosa hereditaria (EBH) [2]. Since the latter term soon became adopted for several congenital traumatic blistering diseases, Hallopeau [3] found it necessary to call the original type’ simple’, i. e. epidermolysis bullosa simplex (EBS). By 1908 EBS was recognized as a dominant Mendelian trait [4]. Incidentally, Kobner’s cases had mainly blisters of the hands and feet with only occasional blisters elsewhere. Many similar families were later called localized EBS (Weber-Cockayne) named after Weber’s (1926) solitary ‘recurrent bullous eruption of the feet’ case [5] and Cockayne’s (1938) two dominant families with blistering feet only [6]. Such cases had previously [7] been called EBS, but Cockayne distinguished them from EBS. Since 1957 [8], EBS (Weber-Cockayne) has been used for cases of blistering of the feet or hands and feet, and EBS (Kobner) for more generalized blistering. One of Cockayne’s families had the additional feature of ‘easily bruising skin’ and may therefore represent a different disease (EBS Ogna) first distinguished in 1970 [9].
广泛性显性单纯性大疱性表皮松解症：作为表型标记的培养成纤维细胞中明胶分解蛋白酶的活性降低。
DOI：
10.1111/1523-1747.ep12523269
发表时间：
1983
期刊：
The Journal of investigative dermatology
影响因子：
--
作者：
Sanchez,G;Seltzer,JL;Eisen,AZ;Stapler,P;Bauer,EA
通讯作者：
Bauer,EA