Basal Cell Nevus Syndrome

Synonyms

Fifth Phacomatoses

Fifth Phacomatosis

Gorlin Goltz Syndrome

Gorlin Syndrome

Gorlin-Goltz Syndrome

Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies

NBCCS

Nevoid Basal Cell Carcinoma Syndrome

Nevus Syndrome, Basal Cell

Syndrome, Gorlin

Syndrome, Gorlin-Goltz

Hereditary disorder consisting of multiple basal cell carcinomas, odontogenic keratocysts, and multiple skeletal defects, e.g., frontal and temporoparietal bossing, bifurcated and splayed ribs, kyphoscoliosis, fusion of vertebrae, and cervicothoracic spina bifida. Genetic transmission is autosomal dominant.