先天性外胚层发育不良症是一种少见的遗传性疾病,国内谭氏(1963年)报告1例为4(4/21)岁男孩,孙氏(1975年)报告7例,年龄3～22岁,均为男性。我院新生儿病室于1977年11月至1978年5月发现2例,报道如下. [例1] 沈姓,男,出生9天。患儿为第四胎第二产,足月顺产。生后第三天喂奶时发现小儿发热,热时高时退,但吮奶和反应均好。于第八天随其母出产院,当晚小儿发热至40℃,于次晨(1977年11月26日)入我院。体检:营养发育正常,头发较稀疏、呈淡黄褐色,眉毛亦稀疏,睫毛缺如。皮肤较干燥,面部、四肢部分表皮皲裂脱屑。眼结膜充血、有少量分泌物,咽不红,心、肺无异常,腹软, Congenital ectodermal dysplasia is an uncommon hereditary disease. One case of 4 (4/21) years old was reported by Tan (1963) in China, 7 cases were reported by Sun (1975), and the age ranged from 3 to 22 Years old, are men. Neonatal ward in our hospital from November 1977 to May 1978 found 2 cases, reported as follows. [Example 1] Shen surname, male, born 9 days. The second child with a fourth child fetus, term full-term. On the third day after giving birth, she found that she had fever when she was breastfeeding, and she retreated when her temperature was high, but her sucking and reaction were good. On the eighth day with his mother’s mother’s abortion, children with fever that night to 40 ℃, the next morning (November 26, 1977) into our hospital. Physical examination: normal development of nutrition, hair more sparse, pale yellow, eyebrows are sparse, lack of eyelashes. Skin is dry, facial, limbs, chapped skin and chapped. Conjunctival hyperemia, a small amount of secretions, pharynx red, heart, lung no abnormalities, abdominal soft,