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遗传性和获得性寻常型鱼鳞病

Hereditary and Acquired Ichthyosis Vulgaris

作者信息

Majmundar Vidit D., Baxi Kalgi

机构信息

Saint Vincent Hospital, Worcester, MA

SMT NHL MEDICAL COLLEGE AND SVP HOSPITAL

Abstract

The term "ichthyosis" implies a generalized scaly dermatosis, without the component of erythema. The ichthyoses are disorders of cornification and are classified as inherited or acquired. Hereditary ichthyosis is further classified as syndromic and non-syndromic. Ichthyosis vulgaris (IV) is an inherited form of non-syndromic ichthyosis, with a very early age of onset. Ichthyosis vulgaris is associated with loss of function FLG gene mutations, which leads to a defective skin barrier. Historically, the first case of ichthyosis vulgaris was described in 1806 as "ichthyose nacrée." Willan introduced the term "ichthyosis," derived from the Greek root "ichthy," meaning fish.

摘要

“鱼鳞病”一词意味着一种全身性的鳞屑性皮肤病,不伴有红斑成分。鱼鳞病是角化异常性疾病,分为遗传性和获得性。遗传性鱼鳞病进一步分为综合征型和非综合征型。寻常型鱼鳞病(IV)是一种遗传性非综合征型鱼鳞病,发病年龄很早。寻常型鱼鳞病与功能缺失性FLG基因突变有关,这会导致皮肤屏障功能缺陷。历史上,寻常型鱼鳞病的首例病例于1806年被描述为“珍珠状鱼鳞病”。威兰引入了“鱼鳞病”这个术语,它源自希腊词根“ichthy”,意为鱼。

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