先天性无牙畸形伴无汗症较为罕见,本病系外胚层发育不全,外胚层结构不良引起退行性发育畸形。患者无牙可包括乳牙列及恒牙列,但亦可仅限于恒牙列。本病常伴有皮肤及其附件缺陷如汗腺、皮脂腺、毛囊部分缺乏或全部缺失。迄今国内外共收集约有300余例,国内报道甚少。笔者自1978年至198t年遇有3病例,现提出初步报告供同道参考并惠予指正。病例介绍:例1 蒲××,男,9岁,学 Congenital tooth-free deformity associated with no more common sweat-less, the disease Department of ectodermal hypoplasia, dysplasia of ectoderm caused degenerative deformity. Patients without teeth can include deciduous teeth and permanent dentition, but may also be limited to permanent dentition. The disease is often accompanied by defects in the skin and its annexes such as sweat glands, sebaceous glands, hair follicles part of the lack of or missing. To date, there are about 300 cases collected at home and abroad, with little domestic coverage. From 1978 to 198t, the author encountered 3 cases, and I present my preliminary report for reference and benefit me. Case description: Example 1 Pu × ×, male, 9 years old, learning