137. Genetic Diseases that Predispose to Malignancy

  1. Alan D. Irvine MD, FRCPI, FRCP2,3,
  2. Peter H. Hoeger MD4,5 and
  3. Albert C. Yan MD, FAAP, FAAD6,7
  1. Julie V. Schaffer MD

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch137

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

Schaffer, J. V. (2011) Genetic Diseases that Predispose to Malignancy, 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.ch137

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. Department of Dermatology, New York University School of Medicine, New York, NY, USA

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:

  • Bannayan–Riley–Ruvalcaba syndrome;
  • Bazex–Dupré–Christol syndrome;
  • Beckwith–Wiedemann syndrome;
  • Birt–Hogg–Dubé syndrome;
  • Brooke–Spiegler syndrome;
  • Cowden syndrome;
  • cylindromatosis;
  • epidermodysplasia verruciformis;
  • Gardner syndrome;
  • leiomyomatosis;
  • Muir–Torre syndrome;
  • constitutional mismatch repair deficiency syndrome;
  • Peutz–Jeghers syndrome;
  • PTEN hamartoma-tumour syndrome;
  • trichoepitheliomas

Summary

Hereditary cancer syndromes that affect the skin can be divided into the following groups: (1) disorders that predispose patients to mucocutaneous malignancies; (2) disorders that predispose patients to mucocutaneous and extracutaneous malignancies; and (3) disorders that have cutaneous stigmata and predispose patients to extracutaneous malignancies. Genodermatoses with malignant potential that are discussed in this chapter include Bazex–Dupré–Christol syndrome, Beckwith–Wiedemann syndrome, Birt–Hogg–Dubé syndrome, Brooke–Spiegler syndrome, epidermodysplasia verruciformis, hereditary leiomyomatosis, Gardner syndrome, Muir–Torre syndrome and constitutional mismatch repair deficiency syndromes, Peutz–Jeghers syndrome, and PTEN hamartoma-tumour syndrome.