神经纤维瘤病包括两种不同类型的遗传异常,是神经皮肤综合征最常见的原因,常表现为多器官受累,但头颅脊柱受累最常见,神经纤维瘤病Ⅰ型(NF-1)最常见,发病率为1/4000,50％的NF-1为基因突变造成,余为常染色体显性遗传,NF-1基因位于染色体17上,临床上有许多表现,其中有皮肤咖啡牛奶斑、神经纤维瘤、丛状神经纤维瘤病、腋窝及腹股沟雀斑、视神经胶质瘤、Lish结 Neurofibromatosis includes two different types of genetic abnormalities. It is the most common cause of neurodermabrasion syndrome. It is often manifested as multiple organ involvement, but the most common involvement of the cranial spine, neurofibromatosis type I (NF-1) is the most common The incidence rate is 1/4000, 50% of NF-1 is caused by gene mutations, and the rest is autosomal dominant. NF-1 gene is located on chromosome 17, and there are many clinical manifestations, including skin coffee milk spots and nerves. Fibroma, plexiform neurofibromatosis, axillary and groin freckles, optic glioma, Lish knot