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Gorlin syndrome

Disease definition

Gorlin syndrome (GS) is a genodermatosis characterized by multiple early-onset basal cell carcinoma (BCC), odontogenic keratocysts and skeletal abnormalities.

ORPHA:377

  • 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

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