先天性外胚层发育不全。是胚胎期外胚层发育障碍引起的一种先天性疾病。主要表现为皮肤及其附属器官如毛发、皮脂腺、汗腺、指(趾)甲以及牙齿的发育异常.本病少见,国外 Secvenson 和 Kerr 估计该病在牛津地区的发生率为十万分之一。国内尚无这方面的统计。现将笔者所遇二例报道如下:例1:男,2.5岁.自生后发热,高热时伴抽风。用各种抗生素及退热剂无效。以后发现发热与环境温度高、穿衣服过多有关,从来不出汗。患儿系第二胎第二产, Congenital ectodermal hypoplasia. Is a congenital disorder caused by the development of embryonic ectoderm. The main manifestations of the skin and its appendages such as hair, sebaceous glands, sweat glands, toenails and dental development abnormalities rare disease, foreign Secvenson and Kerr estimated the incidence of the disease in Oxford is one hundred thousandth. There is no statistics in this area in China. Now the author encountered two cases reported as follows: Example 1: Male, 2.5 years old .After birth, fever, high fever with ventilation. With a variety of antibiotics and antipyretics invalid. After the discovery of fever and the high ambient temperature, excessive wear clothes, never sweating. Pediatric second child second child,