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.
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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