89. Linear IgA Associated Bullous Disease in Children: Childhood Linear IgA Disease/Chronic Bullous Disease of Childhood, Mixed Immunobullous Disease and IgA Mucous Membrane Pemphigoid

  1. Alan D. Irvine MD, FRCPI, FRCP3,4,
  2. Peter H. Hoeger MD5,6 and
  3. Albert C. Yan MD, FAAP, FAAD7,8
  1. Gudula Kirtschig1 and
  2. Fenella Wojnarowska2

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch89

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

Kirtschig, G. and Wojnarowska, F. (2011) Linear IgA Associated Bullous Disease in Children: Childhood Linear IgA Disease/Chronic Bullous Disease of Childhood, Mixed Immunobullous Disease and IgA Mucous Membrane Pemphigoid, 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.ch89

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

    Consultant Dermatologist, VU University Medical Center, Amsterdam, The Netherlands

  2. 2

    Nuffield Department of Medicine, University of Oxford, 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:

  • chronic bullous disease of childhood;
  • linear IgA disease;
  • mixed immunobullous disease;
  • mucous membrane pemphigoid

Summary

Linear immunoglobulin A disease of childhood, also known as chronic bullous disease of childhood, is probably best defined as an acquired blistering disease with involvement of the skin and mucous membranes with blisters and erosions. Linear immunoglobulin A disease is characterized by immunoglobulin A antibodies reacting with the basement membrane zone of the skin in a linear pattern. If mucosal involvement is the dominant feature it is best classified as mucous membrane pemphigoid. The classic presentation is in a preschool child with tense blisters, some arising from urticated plaques and some from normal skin. The mean disease duration is 3–5 years, after which spontaneous remission occurs in the majority of patients. Therapy is aimed at controlling the disease while waiting for spontaneous remission. Potent topical steroids may be beneficial for mucosal lesions, which are often resistant to systemic therapy. The skin blistering may be suppressed by dapsone or sulphonamides.

chronic bullous disease of childhood, linear IgA disease, mixed immunobullous disease, mucous membrane pemphigoid