先天性鱼鳞病,是一种先天性遗传性皮肤病,但并发宫内重度脱水实属罕见,我科近期收治1例,现报告如下.男,因生后27小时无尿而收入我科。系1孕1胎,胎龄40周,胎膜早破,顺产,脐带绕颈一周,羊水清,娩出即时评分(Apgar 法)为9分,体重2.7 Congenital ichthyosis is a congenital hereditary dermatosis, but complicated with intrauterine severe dehydration is rare, our department recently admitted in 1 case, the report is as follows. Male, 27 hours after birth, anuria income in our department. Department of a pregnant and 1 fetus, gestational age 40 weeks, premature rupture of membranes, natural delivery, umbilical cord around the neck a week, clear amniotic fluid, delivered immediate score (Apgar method) was 9 points, weight 2.7