隐性X-链鱼鳞癣(RXLI)合并类固醇硫酸酯酶(STS)缺乏在男性中占1/2500至1/5000。在妊娠中,当STS缺乏时,胎儿以类固醇硫酸盐生成雌激素会非常低下。出生后,在患病男性中,皮肤和血液中有胆固醇硫酸盐的积聚,然而此酶缺乏对于性激素的代谢有何影响则迄今未阐明。Traupe和Happle在25例患RXLI和STS缺乏的病人中发现7例隐睾,故认为他们之间有因果关系。此外,他们复习文献,发 The recessive X-chain ichthyosis (RXLI) combined steroid sulfatase (STS) deficiency accounts for 1/2500 to 1/5000 in men. In pregnancy, when the STS is deficient, fetal production of estrogen from steroid sulfate is very low. After birth, there is accumulation of cholesterol sulfate in the skin and blood in diseased men. However, the effect of this enzyme deficiency on the metabolism of sex hormones has not been elucidated to date. Traupe and Happle found 7 cases of cryptorchidism in 25 patients with RXLI and STS deficiency, so they are considered to have a causal relationship. In addition, they review the literature and send