BASAL CELL NEVUS SYNDROME
Basal cell nevus syndrome (BCNS), also known as nevoid basal cell carcinoma syndrome or Gorlin syndrome, is an uncommon autosomal dominant genodermatosis caused by mutations in the patched-1 (PTCH1) gene on chromosome 9. Approximately 40% of cases represent new, spontaneous mutations. Affected individuals are predisposed to the development of multiple basal cell carcinomas (BCCs), often in the hundreds over their lifetime. The diagnosis of this syndrome is based on a number of established criteria.