Abstract
Background
Juvenile localised scleroderma (JLS) is a rare connective tissue disorder in childhood. Most lesions are benign and self-limiting, but some progress to cause functional disabilities and cosmetic disfigurement. These lesions require systemic treatment, the mainstay of which is corticosteroids and methotrexate.
Aim
To report the experience of the use of systemic treatment in children with JLS in our department.
Methods
We performed a retrospective chart review looking at the patients who received systemic treatment over 14 years and recorded their outcomes.
Results
Ten children with aggressive JLS were treated with systemic immunosuppressive therapy. There was an 80% response to treatment. Three of the responders relapsed off treatment, but responded to a further course of therapy. Mean disease duration at diagnosis was 8 months. Treatment was generally well tolerated with few side effects.
Conclusion
Early recognition of aggressive localised scleroderma and appropriate referral is imperative for a good outcome.
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References
Uziel Y, Feldman BM, Krafchik BR et al (2000) Methotrexate and corticosteroid treatment for pediatric localized scleroderma. J Pediatr 136:91–95. doi:10.1016/S0022-3476(00)90056-8
Weibel L, Sampaio MC, Visentin MT et al (2006) Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma in children. Br J Dermatol 155(5):1013–1020. doi:10.1111/j.1365-2133.2006.07497.x
Zulian F, Athreya BH, Laxer R et al (2006) Juvenile Localized Scleroderma: clinical and epidemiological features in 750 children. Rheumatology 45(5):614–620. doi:10.1093/rheumatology/kei251
Zulian F, Vallongo C, Woo P et al (2005) Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 52:2873–2881. doi:10.1002/art.21264
Kreuter A, Hyun J, Stücker M et al (2006) Randomized controlled study of low-dose UVA1, medium dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol 54(3):440–447. doi:10.1016/j.jaad.2005.11.1063
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Cox, D., O’ Regan, G., Collins, S. et al. Juvenile localised scleroderma: a retrospective review of response to systemic treatment. Ir J Med Sci 177, 343–346 (2008). https://doi.org/10.1007/s11845-008-0217-0
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DOI: https://doi.org/10.1007/s11845-008-0217-0