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Juvenile Localized Scleroderma

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Scleroderma

Abstract

Juvenile localized scleroderma (JLS), known as morphea, comprises a group of conditions which involve essentially the skin and subcutaneous tissues. They have various features and range from very small plaques to extensive fibrotic lesions which may cause significant functional changes and cosmetic deformities.

Although JLS is relatively uncommon, it is far more common than systemic sclerosis in childhood, by a ratio of at least 10:1. The disease onset is usually during late infancy, although cases with onset at early infancy and even at birth have been described.

An accurate clinical examination associated with instrumental assessment with thermography, ultrasonography, and skin scoring is crucial to address the appropriate treatment.

During the last few years, methotrexate (MTX) in combination with corticosteroids has shown to be safe and effective, particularly in patients with significant risk for disability.

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References

  1. Peterson LS, Nelson AM, Su WPD, et al. Subspecialty clinics: rheumatology and dermatology. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995;70:1068–76.

    Article  PubMed  CAS  Google Scholar 

  2. Zulian F, Athreya BH, Laxer RM, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45:614–20.

    Article  CAS  Google Scholar 

  3. Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006;18:606–13.

    Article  PubMed  Google Scholar 

  4. Herrick AL, Ennis H, Bhushan M, et al. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res (Hoboken). 2010;62(2):213–8.

    Google Scholar 

  5. Zulian F, Vallongo C, de Oliveira SKF, et al. Congenital localized scleroderma. J Pediatr. 2006;149:248–51.

    Article  PubMed  Google Scholar 

  6. Jablonska S, Blaszczyk M. Long-lasting follow-up favours a close relationship between progressive facial hemiatrophy and scleroderma en coup de sabre. J Eur Acad Dermatol Venereol. 2005;19:403–4.

    Article  PubMed  CAS  Google Scholar 

  7. Menni S, Marzano AV, Passoni E, et al. Neurologic abnormalities in two patients with facial hemiathrophy and sclerosis coexisting with morphea. Pediatr Dermatol. 1997;14:113–6.

    Article  PubMed  CAS  Google Scholar 

  8. Blaszczyk M, Jablonska S. Linear scleroderma En coup de sabre: relationship with progressive facial hemiatrophy. Adv Exp Med Biol. 1999;455:101–4.

    Article  PubMed  CAS  Google Scholar 

  9. Sommer A, Gambichler T, Bacharach-Buhles M, et al. Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol. 2006;54:227–33.

    Article  PubMed  Google Scholar 

  10. Wollina U, Buslau M, Weyers W, et al. Squamous cell carcinoma in pansclerotic morphea of childhood. Pediatr Dermatol. 2002;19:151–4.

    Article  PubMed  Google Scholar 

  11. Parodi PG, Roberti G, Draganic Stinco D, et al. Squamous cell carcinoma arising in a patient with long-standing pansclerotic morphea. Br J Dermatol. 2001;144:417–9.

    Article  PubMed  CAS  Google Scholar 

  12. Maragh SH, Davis MD, Bruce AJ, et al. Disabling pansclerotic morphea: clinical presentation in two adults. J Am Acad Dermatol. 2005;53:115–9.

    Article  Google Scholar 

  13. Zulian F, Vallongo C, Woo P, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005;52:2873–81.

    Article  PubMed  Google Scholar 

  14. Blaszczyk M, Krolicki L, Krasu M, et al. Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol. 2003;30:1997–2004.

    PubMed  Google Scholar 

  15. DeFelipe J, Segura T, Arellano JI, et al. Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome. Epilepsia. 2001;42:1198–203.

    Article  PubMed  CAS  Google Scholar 

  16. Flores-Alvarado DE, Esquivel-Valerio JA, Garza-Elizondo M, et al. Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic relationship? J Rheumatol. 2003;30:193–5.

    PubMed  Google Scholar 

  17. Holland KE, Steffes B, Nocton JJ, et al. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics. 2006;117:132–6.

    Article  Google Scholar 

  18. Weber P, Ganser G, Frosch M, et al. Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease. J Rheumatol. 2000;27:2692–5.

    PubMed  CAS  Google Scholar 

  19. Guariso G, Conte S, Galeazzi F, et al. Esophageal involvement in juvenile localized scleroderma: a pilot study. Clin Exp Rheumatol. 2007;25:786–9.

    PubMed  CAS  Google Scholar 

  20. Takehara K, Sato S. Localized scleroderma is an autoimmune disease. Rheumatology (Oxford). 2005;44:274–9.

    Article  CAS  Google Scholar 

  21. Ruffatti A, Peserico A, Glorioso S, et al. Anticentromere antibody in localized scleroderma. J Am Acad Dermatol. 1986;15:637–42.

    Article  PubMed  CAS  Google Scholar 

  22. Martini G, Murray KJ, Howell KJ, et al. Juvenile-onset localized scleroderma activity detection by infrared thermography. Rheumatology (Oxford). 2002;41:1178–82.

    Article  CAS  Google Scholar 

  23. Zulian F, Meneghesso D, Grisan E, et al. A new computerized method for the assessment of skin lesions in localized scleroderma. Rheumatology (Oxford). 2007;46:856–60.

    Article  CAS  Google Scholar 

  24. Li SC, Liebling MS, Haines KA, et al. Ultrasonography is a sensitive tool for monitoring localized scleroderma. Rheumatology (Oxford). 2007;46:1316–9.

    Article  CAS  Google Scholar 

  25. Liu P, Uziel Y, Chuang S, et al. Localized scleroderma: imaging features. Pediatr Radiol. 1994;24:207–9.

    Article  PubMed  CAS  Google Scholar 

  26. Zulian F. New developments in localized scleroderma. Curr Opin Rheumatol. 2008;20:601–7.

    Article  PubMed  Google Scholar 

  27. Cunningham BB, Landells ID, Langman C, et al. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol. 1998;39:211–5.

    Article  PubMed  CAS  Google Scholar 

  28. Kerscher M, volkenandt M, Gruss C, et al. Low dose UVA phototherapy for treatment of localized scleroderma. J Am Acad Dermatol. 1998;38:21–3.

    Article  PubMed  CAS  Google Scholar 

  29. Kreuter A, Gambichler T, Avermaete A, et al. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediatr Dermatol. 2001;18:241–5.

    Article  PubMed  CAS  Google Scholar 

  30. Camacho NR, Sánchez JE, Martin RF, et al. Medium-dose UVA1 phototherapy in localized scleroderma and its effect in CD34-positive dendritic cells. J Am Acad Dermatol. 2001;45:697.

    Article  PubMed  CAS  Google Scholar 

  31. De Rie MA, Bos JD. Photochemotherapy for systemic and localized scleroderma. J Am Acad Dermatol. 2000;43:725–6.

    Article  PubMed  Google Scholar 

  32. Kreuter A, Hyun J, Stucker M, et al. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54:440–7.

    Article  PubMed  Google Scholar 

  33. Kreuter A, Hyun J, Skrygan M, et al. Ultraviolet A1-induced downregulation of human beta-defensins and interleukin-6 and interleukin-8 correlates with clinical improvement in localized scleroderma. Br J Dermatol. 2006;155:600.

    Article  PubMed  CAS  Google Scholar 

  34. Staberg B, Wulf HC, Klemp P, et al. The carcinogenic effect of UVA irradiation. J Invest Dermatol. 1983;81:517–9.

    Article  PubMed  CAS  Google Scholar 

  35. Setlow RB, Grist E, Thompson K, et al. Wave-lengths effective in induction of malignant melanoma. Proc Nat Acad Sci USA. 1992;90:6666–70.

    Article  Google Scholar 

  36. Caca-Biljanovska NG, Vlckova-Laskoska MT, Dervendi DV, et al. Treatment of generalized morphea with oral 1,25-dihydroxyvitamin D3. Adv Exp Med Biol. 1999;455:299.

    Article  PubMed  CAS  Google Scholar 

  37. Hulshof MM, Bouwes BJN, Bergman W, et al. Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acad Dermatol. 2000;43:1017.

    Article  PubMed  CAS  Google Scholar 

  38. Seyger MM, de Boo Theo, van den Hoogen FHJ, et al. Low-dose methotrexate in the treatment of widespread morphea. J Am Acad Dermatol. 1998;39:220–5.

    Article  PubMed  CAS  Google Scholar 

  39. Uziel Y, Feldman BM, Krafchik BR, et al. Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr. 2000;136:91–5.

    Article  PubMed  CAS  Google Scholar 

  40. Kreuter A, Gambichler T, Breuckmann F, et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol. 2005;141:847–52.

    Article  PubMed  CAS  Google Scholar 

  41. Fitch PG, Retting P, Burnham JM, et al. Treatment of pediatric localized scleroderma with methotrexate. J Rheumatol. 2006;33:609–14.

    PubMed  CAS  Google Scholar 

  42. Weibel L, Sampaio MC, Visentin MT, et al. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma ­(morphea) in children. Br J Dermatol. 2006;155:1013–20.

    Article  PubMed  CAS  Google Scholar 

  43. Zulian F, Martini G, Vallongo C, et al. Methotrexate in juvenile localized scleroderma: a randomised, double-blind, placebo-controlled trial. Arthritis Rheum. 2011;63(7):1998–2006.

    Article  PubMed  CAS  Google Scholar 

  44. Martini G, Ramanan AV, Falcini F, et al. Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatology (Oxford). 2009;48:1410–3.

    Article  Google Scholar 

  45. Lapiere JC, Aasi S, Cook B, Montalvo A. Successful correction of depressed scars of the forehead secondary to trauma and morphea en coup de sabre by en bloc autologous dermal fat graft. Dermatol Surg. 2000;26:793–7.

    Article  PubMed  CAS  Google Scholar 

  46. Palmero ML, Uziel Y, Laxer RM, et al. En coup de sabre scleroderma and parry-romberg syndrome in adolescents: surgical options and patient-related outcomes. J Rheumatol. 2010;37:2174–9.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pediatrics, University Hospital of Padua, Padua, Italy

    Francesco Zulian MD

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  1. Francesco Zulian MD
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Correspondence to Francesco Zulian MD .

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Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

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

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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Zulian, F. (2012). Juvenile Localized Scleroderma. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_9

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  • DOI: https://doi.org/10.1007/978-1-4419-5774-0_9

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