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.