Basal Cell Nevus Syndrome

**Definition:** 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.

**Tree numbers:** - C04.182.089.530.690.150 - C04.557.470.200.649.002.001 - C04.588.805.511.002.001 - C04.700.175 - C05.116.099.105 - C05.500.470.690.150 - C07.320.450.670.130 - C16.131.077.130 - C16.320.700.175 - C17.800.882.623.002.001

**Synonyms:** - Fifth Phacomatosis - Gorlin Syndrome - Gorlin-Goltz Syndrome - Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies - NBCCS - Nevoid Basal Cell Carcinoma Syndrome - Nevus Syndrome, Basal Cell