Abstract

Objective: Localized scleroderma (LS), which is also called as morphea, is a rare skin disease with unknown etiology. LS is typically characterized by sclerosis in the dermis and the subcutaneous tissue. The number of retrospective studies examining the epidemiological, clinical and laboratory data of patients with juvenile LS in Turkey is very limited. The purpose of this study was to investigate the clinical and demographic characteristics of pediatric patients under the age of 18, who were followed up with a diagnosis LS, also to evaluate and compare these findings with available literature.

Material and Methods: The medical records of 39 patients, who had been clinically and histopathologically diagnosed with LS and followed up in our clinic between 2012-2018, were retrospectively reviewed. Demographic, clinical and laboratory findings, and treatment options of the patients were recorded.

Results: A total of 39 pediatric patients (8 boys, 31 girls, mean age 12.1 years) with LS were enrolled in the present study. The age at disease onset was 8.6 years. The mean duration of the disease was 3.6 years. The most common type was plaque type morphea. In two cases, there was movement restriction in the legs, and lichen sclerosus was concurrently present in another case. 12 patients had antinuclear antibody positivity, while 3 cases had positive Borrelia antibodies.

Conclusion: Morphea has lifelong complications for children. Early diagnosis and monitoring of morphea in the childhood period is important in order to avoid both physical and psychological sequelae that may occur in the future.

Keywords: Epidemiology, Localized scleroderma, Morphea, Pediatric patients

References

  1. 1.Zancanaro PC, Isaac AR, Garcia LT, Costa IM. Localized scleroderma in children: clinical, diagnostic and therapeutic aspects. An Bras Dermatol. 2009;84(2):161-72. PMID: 19503984.
  2. 2.Fett N,Werth VP.Update on morphea: part I. epidemiology, clinical presentation, and
  3. pathogenesis. J Am Acad Dermatol. 2011;64( 2)):217-28; quiz 229-30.PMID.21238823;
  4. doi:10.1016/j.jaad.2010.05.045
  5. 3.Khamaganova I. Localized scleroderma predisposing and trigerring factors. The Open Dermatology Journal.2017;1:11.doi10.2174/1874372201711010001.
  6. 4.Rőcken M and Ghoreschi K. Morphea and lichen sclerosus. In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:1469-76.
  7. 5.Bielsa Marsol I. Update on the classification and treatmentof localized scleroderma. Actas Dermosifiliogr. 2013;104(8):654-66.PMID 23948159;doi10.1016/j.adengl.2012.
  8. 6.Koç S,Uzun S. Skleroderma. Turkiye Klinikleri J Dermatol.-Special Topics 2014;7(3):33-43.
  9. 7.Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. 1997 Jan;24(1):73-80. PubMed PMID: 9002014.
  10. 8.Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006;18(6):606-13. PMID: 17053506; doi:10.1097/01.bor.000245727.40630.c3.
  11. 9 Vallongo C, Woo P, Russo R, Ruperto N, Harper J et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005 Sep;52(9):2873-81. PubMed PMID:16142730.
  12. 10. Murray KJ, Laxer RM. Scleroderma in children and adolescents. Rheum Dis Clin North Am. 2002; 28(3): 603–24 PMID: 12380372.
  13. 11.İzol B, Sarıcaoğlu H, Başkan EB, Toka SO, Adım SB,Aydoğan K, et al. Pediatrik morfea (lokalize skleroderma) 14 olguya ait epidemiyolojik klinik ve laboratuar bulguları. [Pediatric Morfea(Localized Scleroderma)Epidemolojical,clinical and labaratory findings of 14 cases]. Türkderm.. 2011;45(3):132-6.doi:104274/turkderm.72621.
  14. 12.Bulur I, Erdoğan HK, Karapınar T, Saracoglu ZN. Morphea in Middle Anatolia, Turkey: a 5-year single-center experience. .Postepy Allegol.2017;34(4):334-8.PMID:28951708;doi.org/10.51114/ada.2017.69313.
  15. 13.Parlak N, Akay BN, Şanlı HE, Akyol A. Hastalarda klinik özellikler,laboratuvar bulguları,seçilen tedavi yöntemi ve takip sonuçları [The clinical features, laboratoryfindings, treatment and follow-up results of patients with morphea] Turkderm. 2013;47(4):209-13.doi.104274/turkderm.05657.
  16. 14.Zulian F, Athreya BH, Laxer R, Nelson AM. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology(Oxford) .2006;45(5):614-20.PMID: 16368732;doi:10.1093/rheumatology/kei251.
  17. 15.Christen-Zaech S, Hakim MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol. 2008; 59(3): 385–96.PMID: 18571769;doi:10.1016/jaad.2008.05.005.
  18. 16.Mertens JS, Seyger MM, Kievit W, Hoppenreijs EP, Jansen TL, van de Kerkhof PC,et al. Disease recurrence in localized scleroderma: aretrospective analysis of 344 patients with paediatric- or adult-onset disease.Br J Dermatol. 2015 Mar;172(3):722-8. doi: 10.1111/bjd.13514. Epub 2015 Feb 8.PubMed PMID: 25381928.
  19. 17. Marzano AV, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P, et al. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol. 2003 Mar-Apr;13(2):171-6. PubMed PMID: 12695134.
  20. 18. Wu EY, Li SC, Torok KS,Viekud Y, Fuhlbrigge R,Rabinovich E et al. A28: Description of he Juvenile Localized Scleroderma Subgroup of the CARRA Registry. Arthritis Rheumatol (Hoboken, NJ)2014; 66(Suppl 11):S43–4.doi10.1002/art.38444.
  21. 19. Leitenberger JJ, Cayce RL, Haley RW, Adams-Huet B, Bergstresser PR, Jacobe HT. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatriccases. Arch Dermatol. 2009 May;145(5):545-50. doi: 10.1001/archdermatol.2009.79. PubMed PMID: 19451498; PubMed Central PMCID: PMC2938024.
  22. 20.Lis-Święty A, Skrzypek-Salamon A, Ranosz-Janicka I, Brzezińska-Wcisło L.Localized scleroderma: clinical and epidemiological features with emphasis onadulthood- versus childhood-onset disease differences. J Eur Acad DermatolVenereol. 2017;31(10):1595-603. PMID: 28271552.;doi: 10.1111/jdv.14197.
  23. 21.Török E, Ablonczy E. Morphea in children. Clin Exp Dermatol. 1986; 11(6): 607–12.PMID: 3499266.
  24. 22. Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM. Clinical features of childhood localized scleroderma in an incidence cohort. Rheumatology (Oxford).2011 Oct;50(10):1865-8. doi: 10.1093/rheumatology/ker142. Epub 2011 Jul 5. PubMed PMID: 21729934.
  25. 23. Liou JS, Morrell DS.Firm and dyspigmented linear plaques: childhood linear morphea. Pediatri Ann.2007;36(12)792-4.PMID:18229520.
  26. 24.Zulian F.New developments in localized scleroderma. Curr Opin Rheumatol. 2008;20(5):601-7.PMID:18698185.doi:10.109/BOR.0 b013e328309a5eb.
  27. 25. Weber K.Is juvenile localized scleroderma related to Lyme borreliozis? J Am Acad Dermatol. 2009; 61(5):901. PMID:19836648;doi:10.1016/j.jaad.2009.05.022.
  28. 26-Ceylan N, Gürel MS, Kiremitçi Ü, Demirkesen C. Lichen sclero atrophicus in combination with generalized morphea.Türk Patoloji Dergisi. 2008;24(3):179-83 .
  29. 27. Piram M, McCuaig CC, Saint-Cyr C, Marcoux D, Hatami A, Haddad E et al. Short- and long-term outcome of linear morphoea in children. Br J Dermatol. 2013 Dec;169(6):1265-71. doi: 10.1111/bjd.12606. Erratum in: Br J Dermatol. 2014Apr;170(4):999. Dosage error in article text. PubMed PMID: 24032480
  30. 28.Guevara-GutiérrezE,Yinh-LaoJ,García-GutiérrezP,Tlacuilo-ParraA:Frequency of antinuclear antibodies in mestizo Mexican children with morphea. Clin Rheumatol.2010;29(9):1055-9.PMID:20549277;doi:10.1007/s10067-010-1515-2.
  31. 29.Sato S, Fujimoto M, Ihn H, Kikuchi K, Takehara K.Clinical characteristics associated with antihistone antibodies in patients with localized scleroderma. J Am Acad Dermatol. 1994;31(4):567-71.PMID: 8089281.
  32. 30. WooTY,Rasmussen JE.Juvenile linear scleroderma associated with serologic abnormalities Arch Dermatol. 1985;121(11):1403-5. PMID: 3876808.
  33. 31.Alimova E,Farhi D,Plantier F,,et al.Morphea (localizedscleroderma):baseline body surface
  34. involvement and antinuclear antibody may have a prognosticvalue. Clin Exp Dermatol.2009;34(7):e491-2.PMID:19747327;doi:10.1111/j.1365-2230.2009.03557.x.
  35. 32.Fett N, Werth VP. Update on morphea: part II. Outcome measuresand treatment. J Am Acad Dermatol. 2011; 64(2): 231-42. quiz 243-4.PMID:-21238824;doi:10.1016/j.jaad.2010.05.046.
  36. 33.Careta MF, Romiti R. Localized scleroderma: clinical spectrumand therapeutic update. An Bras Dermatol.2015; 90(1):62-73.PMID: 25672301; doi:10.1590/abd1806-4841.20152890.
  37. 34.El-Sakka AI, Bakircioglu ME, Bhatnagar RS,et al.The effects of colchicine on a Peyronie's-like condition in an animal model. J Urol .1999;161(6):1980-3.PMID: 10332485.
  38. 35.Noh JW, Kim J, Kim JW. Localized scleroderma: a clinical study at a single
  39. center in Korea. Int J Rheum Dis. 2013 ;16(4):437-41. PMID: 23992265;doi:10.1111/1756-185X.12080.
  40. 36.Zulian F, Vallongo C, Patrizi A, Belloni-Fortina A, Cutrone M, Alessio M,et al. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea). J Am Acad Dermatol.2012 Dec;67(6):1151-6. doi: 10.1016/j.jaad.2012.03.036. Epub 2012 May 30. PubMed PMID: 22

Copyright and license

How to cite

1.
Akbas A, Kılınç F. Clinical and Demographic Characteristics of Pediatric Patients Diagnosed with Localized Scleroderma: A Retrospective Analysis. Turk J Pediatr Dis [Internet]. 2022 Jan. 28 [cited 2025 May 25];16(1):25-31. Available from: https://turkjpediatrdis.org/article/view/914