Modern data on localized scleroderma and methods of its therapy


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Abstract

Chronic localized scleroderma is a rare disease of the skin and subcutaneous tissue with an unknown etiology and pathogenesis, characterized by a variety of clinical variants. Pathological changes occur in the form of three consecutive stages of erythema or edema, induration and the formation of sclerosis and/or atrophy; however, such staging is not observed in all cases. Localized scleroderma covers a wide range of clinical variants, from solitary skin lesions with minimal discomfort to severe subtypes such as generalized or linear scleroderma. Research on development of a modern classification, clarification of the pathogenesis of the disease and effective methods of therapy is ongoing. Topical glucocorticosteroids are the first-line treatment for localized scleroderma. Ultraviolet A1 (UVA1) phototherapy is used to treat patients with advanced lesions.

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About the authors

Nina N. Filimonkova

Ural Research Institute of Dermatovenereology and Immunopathology

Email: nnfil2008@mail.ru
Dr. Sci. (Med.), Professor, Leading Researcher at the Scientific Clinical Department Yekaterinburg, Russia

A. R Temirbulatova

Doctor Arbitaylo

Tyumen, Russia

I. D Kuznetsov

Academician E.A. Vagner Perm State Medical University

Perm, Russia

References

  1. Peterson L.S., Nelson A.M., Su W.P. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995;70:1068-76. doi: 10.4065/70.11.1068.
  2. Kunzler E., Florez-Pollack S., Teske N., et al. Linear morphea: Clinical characteristics, disease course, and treatment of the Morphea in Adults and Children cohort. J Am Acad Dermatol. 2019;80:1664-70. Doi: 10.1016/j. jaad.2019.01.050.
  3. Teske N., Welser J., Jacobe H. Skin mapping for the classification of generalized morphea. J Am Acad Dermatol. 2018;78:351-57. Doi: 10.1016/j. jaad.2016.08.052.
  4. Mertens J.S., Seyger M.M.B., Thurlings R.M, Radstake T.R.D.J., de Jong EMGJ. Morphea and Eosinophilic Fasciitis: An Update. Am J Clin Dermatol. 2017;18(4):491-512. Doi: 10.1007/ s40257-017-0269-x.
  5. Zulian F., Vallongo C, Woo P., et al., Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society (PRES). Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005;52(9):2873-81. doi: 10.1002/art.21264.
  6. Laxer R.M., Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006;18(6):606-13. doi: 10.1097/01.bor.0000245727.40630.c3
  7. Ihn H., Sato S., Fujimoto M., et al. Demonstration of interleukin-2, interleukin-4 and interleukin-6 in sera from patients with localized scleroderma. Arch Dermatol Res. 1995;287(2):193-97. doi: 10.1007/BF01262331.
  8. Careta M.F., Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol. 2015;90:62-73. Doi: 10.1590/ abd1806-4841.20152890.
  9. Wadud M.A., Bose B.K., Al Nasir T. Familial localised scleroderma from Bangladesh: two case reports. Bangladesh Med Res Counc Bull. 1989;15:15-9.
  10. O'Brien J.C., Rainwater Y.B., Malviya N., et al. Transcriptional and Cytokine Profiles identify CXCL9 as a Biomarker of Disease Activity in Morphea. J Investig Dermatol. 2017;137:1663-doi: 10.1016/j.jid.2017.04.008.
  11. Grabell D., Hsieh C., Andrew R., et al. The role of skin trauma in the distribution of morphea lesions: a cross-sectional survey of the Morphea in Adults and Children cohort IV. J Am Acad Dermatol. 2014;71:493-98. Doi: 10.1016/j. jaad.2014.04.009.
  12. Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011;64:217-228; quiz 229-30. doi: 10.1016/j.jaad.2010.05.045.
  13. Prasad S, Zhu JL, Schollaert-Fitch K, et al. Characterizing morphea subsets using a multicenter, prospective, cross-sectional analysis of morphea in adults and children. J Investig Dermatol. 2020;140:S73.
  14. Badea I., Taylor M., Rosenberg A., et al. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis. Rheumatology (Oxford). 2009;48:213-21. Doi: 10.1093/ rheumatology/ken405.
  15. Kroft E.B., Groeneveld T.J., Seyger M.M., de Jong E.M. Efficacy of topical tacrolimus 0.1% in active plaque morphea: randomized, double-blind, emollient-controlled pilot study. Am J Clin Dermatol. 2009;10(3):181-87. doi: 10.2165/00128071-200910030 00004.
  16. 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(3):440-47. Doi: 10.1016/j. jaad.2005.11.1063.
  17. Joly P, Bamberger N., Crickx B., Belaich S. Treatment of severe forms of localized scleroderma with oral corticosteroids: follow-up study on 17 patients. Arch Dermatol. 1994; 130(5):663-64.
  18. Zulian F., Martini G., Vallongo C., et al. Methotrexate treatment in juvenile localized scleroderma: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2011;63(7):1998-2006. doi: 10.1002/art.30264.
  19. Старовойтова М.Н., Десинова О.В. Опыт применения актовегина при очаговой склеродермии. Современная ревматология. 2013;3:42-

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