Orphanet: Gorlin syndrome

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Gorlin syndrome

Disease definition

A rare hereditary disorder due to autosomal dominant transmission with hamartosis characterized by multiple early-onset basal cell carcinoma (BCC), multiple jaw keratocysts and skeletal abnormalities.


Classification level: Disorder
  • Synonym(s):
    • Basal cell nevus syndrome
    • Gorlin-Goltz syndrome
    • NBCCS
    • Nevoid basal cell carcinoma syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adolescent, Adult
  • ICD-10: Q87.8
  • OMIM: 109400
  • UMLS: C0004779  C0812437
  • MeSH: -
  • GARD: 7166
  • MedDRA: 10062804

Detailed information


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