本病是先天性神经皮肤综合征,发生于胚胎早期。主要病理改变是颅内血管畸形。郭纯钢等认为病因属于母斑病组遗传性病变,可为单纯隐性遗传,相当于胚胎第六周时发生异常所致。据近来遗传学家研究,也认为本病与染色体畸形有关,故又称为“三体-22综合征”。即在第22对染色体上有一个额外染色体。与国外报道相符。国内最早见于1957年马文耕氏报告3例。现国内报道 The disease is congenital neurodermatosis, occurs in early embryos. The main pathological changes are intracranial vascular malformations. Guo Chun believes that the etiology belongs to the mother spot disease group genetic disease, can be a simple recessive, equivalent to the sixth week when the embryo was caused by abnormalities. According to recent studies of geneticists, but also that the disease and chromosomal abnormalities, it is also known as “trisomy 22 syndrome.” That is, there is an extra chromosome on chromosome 22. In line with foreign reports. The earliest in 1957, Ma Wenge reported 3 cases. Now domestic reports