Basal Cell Nevus Syndrome

  • Tor Shwayder
  • Samantha L. Schneider
  • Devika Icecreamwala
  • Marla N. Jahnke


Basal Cell Nevus Syndrome (BCNS) (also known as Gorlin Syndrome) is a rare autosomal dominant disorder associated with basal cell skin cancers and other features. The incidence of BCNS reportedly varies from 1 in 57,000 to 1 in 256,000 [1]. The mutation associated with BCNS is in the PTCH1 gene, a tumor suppressor gene, which plays an important role during embryogenesis affecting cell proliferation, differentiation and cell fate. PTCH mutations have high penetrance but variable phenotypic expression [1].


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Tor Shwayder
    • 1
  • Samantha L. Schneider
    • 2
  • Devika Icecreamwala
    • 3
  • Marla N. Jahnke
    • 2
  1. 1.Pediatric DermatologyHenry Ford HospitalDetroitUSA
  2. 2.Department of DermatologyHenry Ford HospitalDetroitUSA
  3. 3.Icecreamwala DermatologyBerkeleyUSA

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