91. Pemphigus, Pemphigoid and Epidermolysis Bullosa Acquisita

  1. Alan D. Irvine MD, FRCPI, FRCP3,4,
  2. Peter H. Hoeger MD5,6 and
  3. Albert C. Yan MD, FAAP, FAAD7,8
  1. J. Henk Sillevis Smitt1 and
  2. Marcel F. Jonkman2

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch91

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

Sillevis Smitt, J. H. and Jonkman, M. F. (2011) Pemphigus, Pemphigoid and Epidermolysis Bullosa Acquisita, 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.ch91

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 Pediatric Dermatology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

  2. 2

    Department of Dermatology, University Medical Centre Groningen, Groningen, The Netherlands

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:

  • antibodies;
  • chronic benign familial pemphigus;
  • epidermal basement membrane zone;
  • epidermal cell adhesion;
  • epidermolysis bullosa acquisita;
  • pemphigoid;
  • pemphigus;
  • sequelae;
  • skin blisters

Summary

In children the rare autoimmune skin diseases pemphigus, pemphigoid and epidermolysis bullosa acquisita and their variants may occur. Unfortunately, delay in diagnosis occurs frequently. In these dermatoses, antibodies are formed against one of the antigens involved in cell adhesion within either intercellular epidermal cells or the epidermal basement membrane zone. This antibody formation eventually leads to blistering of the skin and/or the stratified epithelium of mucous membranes. In children with these diseases, systemic treatment with prednisone is often necessary to induce remission of the disease; in serious cases, the addition of azathioprine, cyclophosphamide, intravenous immunoglobulines or rituximab may be needed. In some cases, topical corticosteroids or oral dapsone suffice. In most cases the prognosis quoad vitam is good, but the general prognosis is variable and sometimes lifelong treatment may be necessary.