125. Darier Disease

  1. Alan D. Irvine MD, FRCPI, FRCP3,4,
  2. Peter H. Hoeger MD5,6 and
  3. Albert C. Yan MD, FAAP, FAAD7,8
  1. Susan M. Burge OBE, BSc (Bristol), BM BCh, DM (Oxon), FRCP1,2

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch125

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

Burge, S. M. (2011) Darier Disease, 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.ch125

Editor Information

  1. 3

    Trinity College, Dublin, Ireland

  2. 4

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

  3. 5

    University of Hamburg, Hamburg, Germany

  4. 6

    Catholic Children's Hospital Wilhelmstift, Hamburg, Germany

  5. 7

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

  6. 8

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

Author Information

  1. 1

    Department of Dermatology, Oxford Radcliffe Hospitals NHS Trust, Oxford, UK

  2. 2

    Oxford University, Oxford, 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:

  • acantholysis;
  • ATP2A2;
  • Darier disease;
  • dyskeratosis;
  • retinoids;
  • SERCA2

Summary

Darier disease is a rare inherited dermatosis characterised histologically by focal acantholysis, dyskeratosis and hyperkeratosis. The greasy, crusted papules develop predominantly in a seborrhoeic distribution. Inheritance is autosomal dominant. The defective gene in Darier disease encodes the p-type cation pump, sarco-endoplasmic reticulum Ca2+-ATPase isoform 2. Management is challenging but oral retinoids are the most effective therapy in severe disease.