Advertisement

Localized Scleroderma of the Face

  • Francesco ZulianEmail author
  • Sabina Trainito
  • Anna Belloni-Fortina
Chapter

Abstract

Localized scleroderma (LS), also known as morphea, is the most frequent form of scleroderma in childhood and is grouped into five subtypes: circumscribed morphea, linear scleroderma, generalized morphea, pansclerotic morphea, and a mixed subtype, where a combination of two or more of the previous subtypes is present. Linear scleroderma is the most frequent subtype in childhood, and when it involves the face (LSF), it may cause aesthetic and functional abnormalities, sometimes complicated by hemifacial atrophy.

An early diagnosis, an appropriate assessment, and an effective treatment may slow the disease progression and prevent ophthalmological and neurological complications.

Studies over the past few years have added interesting contributions on new outcome measures for the disease assessment and monitoring. Indeed, a recent randomized placebo-controlled trial has confirmed the important role of methotrexate for this particular subtype of scleroderma.

Keywords

Mycophenolate Mofetil Lichen Sclerosus Erythema Migrans Localize Scleroderma Mixed Subtype 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006;18:606.PubMedCrossRefGoogle Scholar
  2. 2.
    Zulian F. Systemic sclerosis and localized scleroderma in childhood. Rheum Dis Clin North Am. 2008;34:239.PubMedCrossRefGoogle Scholar
  3. 3.
    Zulian F. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45:614.CrossRefGoogle Scholar
  4. 4.
    Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007;56:257.PubMedCrossRefGoogle Scholar
  5. 5.
    Zannin ME, et al. Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol. 2007;91:131.CrossRefGoogle Scholar
  6. 6.
    Zulian F, et al. Localized Scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005;52:2873.PubMedCrossRefGoogle Scholar
  7. 7.
    Blaszczyk M, et al. Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol. 2003;30:1997.PubMedGoogle Scholar
  8. 8.
    Sommer A, et al. Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol. 2006;54:227.PubMedCrossRefGoogle Scholar
  9. 9.
    Jablonska S, Blaszczyk M. Scleroderma overlap syndromes. Adv Exp Med Biol. 1999;455:85.PubMedCrossRefGoogle Scholar
  10. 10.
    Tuffanelli DL. Localized scleroderma. Semin Cutan Med Surg. 1998;17:27.PubMedCrossRefGoogle Scholar
  11. 11.
    Vierra E, Cunningham BB. Morphea and localized scleroderma in children. Semin Cutan Med Surg. 1999;18:210.PubMedCrossRefGoogle Scholar
  12. 12.
    Arkachaisri T, et al. The localized scleroderma skin severity index and physician global assessment of disease activity: a work in progress toward development of localized scleroderma outcome measures. J Rheumatol. 2009;36:2819.PubMedCrossRefGoogle Scholar
  13. 13.
    Li SC, Liebling MS. The use of Doppler ultrasound to evaluate lesions of localized scleroderma. Curr Rheumatol Rep. 2009;11:205.PubMedCrossRefGoogle Scholar
  14. 14.
    Martini G, et al. Juvenile-onset localized scleroderma activity detection by infrared thermography. Rheumatology (Oxford). 2002;41:1178.CrossRefGoogle Scholar
  15. 15.
    Weibel L, et al. Laser Doppler flowmetry for assessing localized scleroderma in children. Arthritis Rheum. 2007;56:34.CrossRefGoogle Scholar
  16. 16.
    Zulian F, et al. A new computerized method for the assessment of skin lesions in localized scleroderma. Rheumatology (Oxford). 2007;46:856.CrossRefGoogle Scholar
  17. 17.
    Kreuter A, Altmeyer P, Gambichler T. Treatment of localized scleroderma depends on the clinical subtype. Br J Dermatol. 2007;156:1362.CrossRefGoogle Scholar
  18. 18.
    Zulian F, et al. Methotrexate treatment in juvenile localized scleroderma. A randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2011;63:1998.PubMedCrossRefGoogle Scholar
  19. 19.
    Li SC, et al. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res. 2012;64:1175.Google Scholar
  20. 20.
    Zulian F, et al. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea). J Am Acad Dermatol. 2012;67:1151.PubMedCrossRefGoogle Scholar
  21. 21.
    Martini G, et al. Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatology (Oxford). 2009;48:1410.CrossRefGoogle Scholar
  22. 22.
    Orzechowski NM, et al. Health-related quality of life in children and adolescents with juvenile localized scleroderma. Rheumatology (Oxford). 2009;48:670.CrossRefGoogle Scholar
  23. 23.
    Palmero ML, et al. En coup de sabre scleroderma and Parry-Romberg syndrome in adolescents: surgical options and patient-related outcomes. J Rheumatol. 2010;37:2174.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2014

Authors and Affiliations

  • Francesco Zulian
    • 1
    Email author
  • Sabina Trainito
    • 2
  • Anna Belloni-Fortina
    • 3
  1. 1.Pediatric Rheumatology Unit, Department of Pediatrics- Rheumatology SectionUniversity of PaduaPaduaItaly
  2. 2.Pediatric Rheumatology UnitUniversity of PaduaPaduaItaly
  3. 3.Pediatric Dermatology – Internal MedicineAzienda Ospedaliera – Università di PadovaPadovaItaly

Personalised recommendations