Juvenile localised scleroderma: a retrospective review of response to systemic treatment.

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.