132. Gorlin (Naevoid Basal Cell Carcinoma) Syndrome

  1. Alan D. Irvine MD, FRCPI, FRCP2,3,
  2. Peter H. Hoeger MD4,5 and
  3. Albert C. Yan MD, FAAP, FAAD6,7
  1. Peter A. Farndon MSc, MD, FRCP, DCH

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch132

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

How to Cite

Farndon, P. A. (2011) Gorlin (Naevoid Basal Cell Carcinoma) Syndrome, in Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition (eds A. D. Irvine, P. H. Hoeger and A. C. Yan), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444345384.ch132

Editor Information

  1. 2

    Trinity College, Dublin, Ireland

  2. 3

    Our Lady's Children's Hospital, Dublin, Ireland

  3. 4

    University of Hamburg, Hamburg, Germany

  4. 5

    Catholic Children's Hospital Wilhelmstift, Hamburg, Germany

  5. 6

    University of Pennsylvania School of Medicine, Philadelphia, PA, USA

  6. 7

    The Children's Hospital of Philadelphia, Philadelphia, PA, USA

Author Information

  1. West Midlands Regional Clinical Genetics Service, Birmingham Women's Hospital, Birmingham, UK

Publication History

  1. Published Online: 24 MAY 2011
  2. Published Print: 3 JUN 2011

ISBN Information

Print ISBN: 9781405176958

Online ISBN: 9781444345384

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Keywords:

  • Gorlin (naevoid basal cell carcinoma) syndrome;
  • Gorlin syndrome components - multiple basal cell carcinomas (BCCs);
  • skeletal signs of syndrome - in Egyptian mummies;
  • Gorlin syndrome, and patched (PTCH) gene - at 9q22.3;
  • patched gene and hedgehog signalling pathway - PTCH gene, positional cloning;
  • spectrum of mutations, human PTCH gene - 23 exons, 62 kb of genomic DNA;
  • HH–PTCH–SMO pathway - from Drosophila, mouse and human;
  • adult patient with Gorlin syndrome - facial features, and arched eyebrows;
  • naevi and basal cell carcinomas - as ‘naevi’ and BCCs, histologically identical;
  • jaw cysts, proliferating dental lamina - and satellite cysts, fibrous wall of cyst

Summary

This chapter contains sections titled:

  • References