先天性外胚叶缺陷(Congenital Ecto-dermal Defect)又叫家族性外胚叶发育不良、外胚层发育不全症、外胚层结构不良等。本病于1838年首先由Widderburn氏报告。临床较为罕见,笔者最近发现一例,报告如下。病例报告患儿,男,5岁,生于哈尔滨市。病史:患儿自出生至今从不出汗,不耐热(指一般人可以耐受的气温),有时体温可因外界环境 Congenital ectodermal defects (Congenital Ecto-dermal Defect), also known as familial ectodermal dysplasia, ectodermal hypoplasia, ectodermal dysplasia and so on. The disease was first reported by Widderburn in 1838. Clinical is relatively rare, I recently found a case, the report is as follows. Case report Children, male, 5 years old, was born in Harbin. History: children since birth never sweat, not heat (referring to the average person can tolerate the temperature), and sometimes body temperature due to the external environment