Basal Cell Nevus Syndrome

Basalcellsnevussyndrom

Svensk definition

Ärftlig rubbning bestående av multipla basalcellskarcinom, odontogena keratocystor och multipla skeletala defekter. Autosomal ärftlighetsgång.

Engelsk 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.

Svenska synonymer

Gorlins syndrom

Engelska synonymer

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