Basal Cell Nevus Syndrome in a Family

Abstract

Basal cell nevus syndrome is also named Nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin syndrome. Multiple organ systems may be involved, including skin, skeletal system, genitourinary system and Cell Nevus Syndrome (CNS).We reported two familial cases of basal cell nevus syndrome. The initial presentation was multiple cysts in mandible and maxilla. There were also some skeletal abnormalities in ribs or spine such as hemiveterbra, fusion, hypoplasia and fork of the ribs. After correlated with family history, basal cell nevus syndrome was considered.