173. Morphoea (Localized Scleroderma)
- Alan D. Irvine MD, FRCPI, FRCP3,4,
- Peter H. Hoeger MD5,6,
- Albert C. Yan MD, FAAP, FAAD7,8
Published Online: 24 MAY 2011
DOI: 10.1002/9781444345384.ch173
Copyright © 2011 Blackwell Publishing Ltd
Book Title
Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition
Additional Information
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
- 3
Trinity College, Dublin, Ireland
- 4
Our Lady's Children's Hospital, Dublin, Ireland
- 5
University of Hamburg, Hamburg, Germany
- 6
Catholic Children's Hospital Wilhelmstift, Hamburg, Germany
- 7
University of Pennsylvania School of Medicine, Philadelphia, PA, USA
- 8
The Children's Hospital of Philadelphia, Philadelphia, PA, USA
Publication History
- Published Online: 24 MAY 2011
- Published Print: 3 JUN 2011
ISBN Information
Print ISBN: 9781405176958
Online ISBN: 9781444345384
- Summary
- Chapter
- References
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.