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Basal Cell Nevus Syndrome

definition

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

synonyms

Basal Cell Nevus Syndrome, Gorlin-Goltz Syndrome, Fifth Phakomatosis, Nevoid Basal Cell Carcinoma Syndrome

UMLS

Basal cell carcinoma syndrome, Basal cell naevus syndrome, Basal Cell Nevus Syndrome, Gorlin Syndrome, Gorlin-Goltz syndrome, Gorlin's syndrome, Naevoid basal cell carcinoma syndrome, Naevoid BCC syndrome, Nevoid Basal Cell Carcinoma Syndrome, Nevoid BCC syndrome, Nevus Syndrome, Basal Cell, Syndrome, Gorlin

images

6 images found for this diagnose localisation: palms, diagnosis: Basal Cell Nevus Syndrome diagnosis: Basal Cell Nevus Syndrome localisation: chest, diagnosis: Basal Cell Nevus Syndrome, Superficial Basal Cell Carcinoma localisation: cheek, nose, diagnosis: Basal Cell Nevus Syndrome localisation: forehead, diagnosis: Basal Cell Nevus Syndrome localisation: back, diagnosis: Basal Cell Nevus Syndrome