173. Morphoea (Localized Scleroderma)

  1. Alan D. Irvine MD, FRCPI, FRCP3,4,
  2. Peter H. Hoeger MD5,6 and
  3. Albert C. Yan MD, FAAP, FAAD7,8
  1. Lisa Weibel MD1 and
  2. John Harper MD, FRCP, FRCPCH2

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch173

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

Weibel, L. and Harper, J. (2011) Morphoea (Localized Scleroderma), 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.ch173

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

    University Children's Hospital Zürich, Zürich, Switzerland

  2. 2

    Great Ormond Street Hospital for Children, NHS Trust, London, 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:

  • Localized,scleroderma;
  • morphoea en coup de sabre;
  • Parry–Romberg syndrome;
  • fibrosis;
  • sclerosis;
  • Blaschko's lines;
  • thermography;
  • methotrexate;
  • corticosteroids

Summary

Morphoea or localized scleroderma is a connective tissue disorder characterized by sclerosis of the skin, often affecting underlying subcutaneous tissue, muscle and bone. Although a proportion of patients may have extracutaneous involvement, secondary transformation into systemic sclerosis is exceptional. Morphoea usually begins in childhood and there is a wide variation in its clinical presentation. The linear variant is the most common subtype found in children and is associated with a progressive course and increased risk of complications. Morphoea may progress over years and result in severe functional and cosmetic disability. A genetic background, autoimmune activity and various triggers such as trauma, vaccinations and infections are relevant pathogenic factors. Morphoea often remains unrecognized for many months to years, leading to substantial delay in treatment. The combination of systemic corticosteroids and methotrexate has been established as an effective and well tolerated first-line therapy for progressive morphoea in childhood.