Studies on the pathogenesis of epidermolysis bullosa.
Studies on the pathogenesis of epidermolysis bullosa.
复制标题
大疱性表皮松解症发病机制的研究。
DOI：
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发表时间：
1962
影响因子：
6.5
通讯作者：
R. Pearson
中科院分区：
文献类型：
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作者：
R. Pearson
大疱性表皮松解组疾病的临床分类导致在阐明这些条件下水疱形成的机制方面进展甚微。组织学研究，虽然有相当大的兴趣，但一直是争议的来源，特别是关于弹性组织在这些疾病中的作用。两个因素似乎是造成不同工作者之间结果差异的主要原因。首先是使用不合适的标本。在大多数研究中，对年龄不确定的自然产生的水疱或死后皮肤进行了检查。其次，光学显微镜的分辨率受到限制。在本研究中，我们试图通过专门使用从非起泡或实验起泡区域获得的标本，并通过电子显微镜观察补充光学显微镜检查来减少这些困难。这种方法提供的数据在一定程度上可以定位导致水疱形成的缺陷部位。也可以对这些缺陷的性质作一些谨慎的推测。在其他报道中（1,2），单纯大疱性表皮松解性水泡是由于基底细胞和基底上细胞的解体而产生的。为了方便参考，本文的结果部分（图25）附有一个完全形成的单纯大疱性表皮松解症的电子显微照片，但主要关注的是遗传性叶状表皮松解症、营养不良大疱性表皮松解症（隐性型）和迟发性皮肤卟啉症。表1给出了大疱性表皮松解症的工作分类。
Clinical classification of the diseases in the epidermolysis bullosa group has led to little progress toward elucidation of the mechanisms of blister formation operative in these conditions. Histologic studies, although of considerable interest, have been the source of controversy, particularly as regards the role of the elastic tissue in these diseases. Two factors seem to be responsible for most of the variance in results between different workers. The first of these is use of unsuitable specimens. In most studies, naturally occurring blisters of uncertain age, or post mortem skin was examined. Secondly, there was the limitation iii resolution imposed by use of the light microscope. In the present study attempts were made to reduce these difficulties by the exclusive use of specimens obtained from non-blistered or experimentally blistered areas, and by supplementing light microscope examinations by electron microscope observations. This approach has given data which tends to localize to some extent the site of the defects responsible for blister formation. Some cautious speculations concerning the nature of these defects can also be made. In other reports (1, 2) epidermolysis bullosa simplex blisters were shown to have developed as a result of disintegration of basal and suprabasal cells. For convenient reference an electron micrograph of a fully formed blister of epidermolysis bullosa simplex is included at the end of the Results section of this paper (Fig. 25), but primary concern is given to epidermolysis bullosa hereditaria letalis, epidermolysis bullosa dystrophica (recessive type), and porphyria cutanea tarda. A working classification of the epidermolysis bullosa group of diseases is given in Table I.