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Localized Scleroderma in a Child

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Case Studies in Systemic Sclerosis

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Abstract

Localized scleroderma (morphea) is much more common than systemic sclerosis (SSc) in childhood and is classified based on the Pediatric Rheumatology European Society Consensus meeting into circumscribed, linear (may involve extremity or head) generalized, pansclerotic, and mixed. Linear is the most common form in childhood. While plaque morphea rarely causes significant morbidity, linear lesions of the extremity may lead to limb length discrepancies, muscle wasting, and severe joint contractures. Linear lesions of the head may be associated with significant cosmetic deformity and rarely, seizure disorder. Pansclerotic morphea is associated with significant mortality. In addition to the skin abnormalities, extracutaneous features occur in up to 20% of patients. It has been difficult to assess disease activity and damage, and recently, a number of groups have attempted to come up with new ways to measure these dimensions. Improved outcomes have been reported with a number of agents, most commonly a combination of corticosteroids and methotrexate given by a variety of protocols. Standardization of treatment protocols will be very helpful and leads to a better understanding of treatment outcomes.

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Author information

Authors and Affiliations

  1. Division of Rheumatology, Department of Paediatrics, University of Toronto, The Hospital for Sick Children, 555 University Ave, Toronto, ON, Canada, M5G 1X8

    Ronald M. Laxer

  2. Section of Dermatology, Department of Paediatrics, The Hospital for Sick Children, Toronto, ON, Canada

    Elena Pope M.D., M.Sc.

  3. Department of Rehabilitation Services, The Hospital for Sick Children, Toronto, ON, Canada

    Christine O’Brien M.Sc., B.Sc. Occupational Therapy

Authors
  1. Ronald M. Laxer
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  2. Elena Pope M.D., M.Sc.
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  3. Christine O’Brien M.Sc., B.Sc. Occupational Therapy
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Editor information

Editors and Affiliations

  1. , Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, 29403, USA

    Richard M. Silver

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

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© 2011 Springer-Verlag London Limited

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Laxer, R.M., Pope, E., O’Brien, C. (2011). Localized Scleroderma in a Child. In: Silver, R., Denton, C. (eds) Case Studies in Systemic Sclerosis. Springer, London. https://doi.org/10.1007/978-0-85729-641-2_5

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  • DOI: https://doi.org/10.1007/978-0-85729-641-2_5

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  • Publisher Name: Springer, London

  • Print ISBN: 978-0-85729-640-5

  • Online ISBN: 978-0-85729-641-2

  • eBook Packages: MedicineMedicine (R0)

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