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在胚胎发育时期神经系统、皮肤和眼的发育均来自于外胚叶组织,因此,外胚叶发育的异常可导致神经系统、皮肤和眼同时出现病变,并且来自于中胚叶和内胚叶的组织如心、肺、肾、骨和胃肠等也可有不同程度受累,因此,外胚叶组织发生病变时往往涉及许多系统和器管,临床表现差异性极大,诊断上有一定的困难,故过去对神经皮肤综合征的发病率常估计过低。神经皮肤综合征的病因和发病机制迄今未明,但其中多数具有遗传学特征及家族发病倾向。有一较高的、不完全外现率。1923年Van Der Hoeve氏所命名的“母斑细胞病”
During the embryonic development nervous system, skin and eye development are derived from the ectodermal tissue, therefore, the abnormal development of ectoderm can lead to nervous system, skin and eye lesions simultaneously, and from the mesoderm and endoderm Of organizations such as heart, lung, kidney, bone and gastrointestinal and other may also have varying degrees of involvement, therefore, the occurrence of ectodermal tissue lesions often involve many systems and devices, the clinical manifestations of great differences in the diagnosis of a certain Difficulties, so the past, the incidence of neurological skin syndrome is often underestimated. The etiology and pathogenesis of neurodermatosis have not been known yet, but most of them have genetic characteristics and familial tendency. There is a higher, incomplete rate of appearance. 1923 Van Der Hoeve’s name of “mother cell disease”