基底细胞痣综合征(basal cell neVus syndome,BCNS,痣样基底细胞癌综合征,痣样基底细胞上皮瘤综合征)于1964年由Clendenning等首先报导.因1965年Gorlin等亦曾以“多发性BCNS”为名报导,故又称Gorlin综合征.该病是一种罕见的遗传性多器官疾病,以多发性基底细胞上皮瘤、手足点状凹陷、颌骨囊肿、骨骼发育异常和异位钙化为突出特点.80年代以来,国外对BCNS的研究有了较大的进展,综述如下. The basal cell nevus syndome (BCNS) was first reported by Clendenning et al. in 1964. Since 1965, Gorlin et al. “BCNS” is the name reported, it is also called Gorlin syndrome. The disease is a rare hereditary multi-organ disease, with multiple basal cell epithelial tumors, punctate depressions, jaw cysts, skeletal dysplasia and ectopic calcification. In order to highlight the features, since the 1980s, foreign research on BCNS has made great progress, summarized as follows.