[Anhidrotic ectodermal dysplasia: "congenital ameibomia"].
[Anhidrotic ectodermal dysplasia: "congenital ameibomia"].
复制标题
[无汗性外胚层发育不良：“先天性变形虫病”]。
DOI：
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发表时间：
2007
期刊：
影响因子：
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通讯作者：
J.
中科院分区：
文献类型：
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作者：
J. Allali;O. Roche;D. Monnet;A. Brezin;G. Renard;J.
无汗性外胚层发育不良是一种先天性、通常为 X 连锁的皮肤病，与面部畸形、身材矮小和严重睑缘炎有关。皮肤的异常是表皮异常；真皮腺体减少，特别是汗腺减少，解释了少汗症；甲发育不良;毛发发育不良;以及牙列异常。眼科表现是睑板腺异常，睑板腺减少或缺失，Moll 和 Zeis 腺功能障碍，导致慢性鳞状睑缘炎和泪点闭锁。这些异常会导致眼表的严重攻击，在第二个十年中发生，这通常会导致患者失效，需要严格的随访以避免角膜并发症。
Anhidrotic ectodermal dysplasia is a congenital, generally X-linked dermatosis that associates facial dysmorphy, short stature, and severe blepharitis. The anomalies of the skin are epidermic abnormalities; reduction of the glands of the derm, particularly the sweat glands, explaining the hypohidrosis; onychodysplasia; trichodysplasia; and abnormal dentition. The ophthalmologic manifestations are palpebral anomalies with a reduction in or an absence of Meibomian glands, dysfunction of the Moll and Zeis glands, leading to chronic squamous blepharitis and lacrimal punctal atresia. These anomalies result in severe attacks of the ocular surface, developing during the second decade, which are often invalidating and require a rigorous follow-up to avoid corneal complications.