Pipa Vallejo Adolfo, López Arranz Monje Elena, González García Manuel, Martínez Fernández Miguel, Blanco Moreno Alvarez Buylla Fernando
School of Dentistry, University of Oviedo, Spain.
Med Oral Patol Oral Cir Bucal. 2008 Feb 1;13(2):E119-23.
Ectodermal dysplasias form part of a wide range of syndromes presenting abnormal development of two or more tissues derived from the ectoderm. Hypohidrotic ectodermal dysplasia is a congenital syndrome, characterized by hypotrichosis (hair is sparse, fine and weak; anomalies in the skin and nails), hypohidrosis (due to the paucity of sweat glands which in turn gives rise to sweat disorders) and hypodontia (partial, and occasionally total, absence of primary and/or permanent dentition). A case of a child with hypohidrotic ectodermal dysplasia with oligodontia and marked resorption of the maxillary and mandibular alveolar ridges is presented. A prosthetic rehabilitation in the form of a removable acrylic prosthesis was made, achieving excellent esthetics, functionality and adaptation, thanks to which a considerable improvement in self-esteem has been obtained.
外胚层发育异常是一系列综合征的一部分，这些综合征表现为源自外胚层的两个或更多组织的异常发育。少汗型外胚层发育异常是一种先天性综合征，其特征为毛发稀少（头发稀疏、纤细且脆弱；皮肤和指甲异常）、少汗（由于汗腺稀少，进而导致出汗障碍）和牙发育不全（乳牙和/或恒牙部分缺失，偶尔全部缺失）。本文介绍了一例患有少汗型外胚层发育异常伴牙缺失及上颌和下颌牙槽嵴明显吸收的儿童病例。制作了可摘丙烯酸义齿形式的修复体，获得了极佳的美学效果、功能和适配性，患者的自尊心因此得到了显著提升。
