Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D-3 (Calcitriol) in seven children

Linear scleroderma is a connective tissue disorder that characteristically involves the skin. Skin induration and pigmentary changes present in a line ar distribution, Severe functional and cosmetic disability may occur, espec ially in growing children. No effective therapy for the fibrotic stage of s cleroderma is available at present. Recently a beneficial effect of oral 1, 25-dihydroxyvitamin D-3 (calcitriol) treatment was reported in adults, Calc itriol has a dose-dependent inhibition on fibroblast proliferation and coll agen synthesis and has immunoregulatory activities. We assessed the efficac y of oral calcitriol treatment in seven pediatric patients with linear scle roderma. During the treatment dietary calcium intake was restricted. Calciu m, inorganic phosphate, creatinine, and urea in the serum and urine was mon itored. The urinary calcium:creatinine ratio was measured. The effects of t he treatment were evaluated using a clinical scoring system, No side effect s were observed. Five of the seven patients showed a good to excellent impr ovement of their lesions, One of them partly relapsed after 19 months, but showed an excellent response to a second therapy session with calcitriol. O ne patient with rapidly progressive disease failed to respond to therapy. O ur results indicate that calcitriol can be an effective agent for treating localized scleroderma in children.