先天性外胚层发百不良综合征也称Christ-Siemens综合征，是由于遗传或遗传因素引起的一组综合征，以汗腺和其他附件的部分或全部缺损及无牙齿为特征，是一十分罕见的疾病，出生时的发病率约为十万分之一[1]。临床上表现为全身不出汗、无毳毛和毛孔、毛发稀少、牙齿缺损等。由于 Congenital ectodermal syndrome, also known as Christ-Siemens syndrome, is a group of syndromes caused by genetic or genetic factors that are characterized by partial or complete absence of sweat glands and other attachments and toothlessness Rare disease, the incidence of birth is about one hundred thousandth [1]. Clinical manifestations of the body does not sweat, no beard and pores, hair scarce, tooth defects and so on. due to