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外胚层发育异常。分类问题及一些新综合征

The ectodermal dysplasias. Problems of classification and some newer syndromes.

作者信息

Solomon L M, Keuer E J

出版信息

Arch Dermatol. 1980 Nov;116(11):1295-9. doi: 10.1001/archderm.116.11.1295.

DOI:10.1001/archderm.116.11.1295
PMID:7002047
Abstract

The ectodermal dysplasias are a heterogeneous group of disorders that, in the past, has included conditions best classified as progeroid disorders. The inaccuracy of the terminology has led to a proliferation of syndromes in which the patients are said to have poorly defined "ectodermal dysplasia," and a real need exists to define that appellation further. We suggest that the term "ectodermal dysplasia" be limited to those disorders that are congenital, are diffusely present, are not progressive, and do involve the epidermis and at least one of the appendages. We recognize that a heterogeneous group of disorders remains that generically have certain similarities. Not enough is known about the defects in each of the elements of the skin affected in these conditions to classify them more accurately. Several recently described disorders appear to have some degree of ectodermal dysplasia.

摘要

外胚层发育不良是一组异质性疾病,过去曾包括一些最好归类为早老样疾病的病症。术语的不准确导致了一系列综合征的增多,在这些综合征中,患者被认为患有定义不明确的“外胚层发育不良”,因此确实有必要进一步明确该称谓。我们建议将“外胚层发育不良”这一术语仅限于那些先天性、广泛存在、非进行性且确实累及表皮和至少一种附属器的疾病。我们认识到仍存在一组异质性疾病,它们总体上有某些相似之处。对于这些病症中受影响的皮肤各要素的缺陷了解不足,无法更准确地对它们进行分类。最近描述的几种病症似乎有一定程度的外胚层发育不良。

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