Sklerodermie en coup de sabre: Erfolgreiche Therapie mit topischem Calcipotriol und Medium-Dose UV-A1-Phototherapie

We report on a 13-year old adolescent female patient with localized scleroderma en coup de sabre. The disease continuously progressed since the age of 11 despite treatments with topical corticosteroids, heparinoids and local creme PUVA phototherapy. We treated with a combination of topical calcipotriol 0.005% twice a day and 30 daily whole-body irradiations each of 30 J/cm 2 UVA-1. After that treatment a stable stop of the progress and a softening of the lesion was achieved. Furthermore, 20 MHz-ultrasound density measurements of the lesional skin showed a marked decrease from 47 to 24 relative units (compared to 14 units in uninvolved contralateral skin). The slightly decreased skin thickness and the markedly decreased subcutis thickness did not change during therapy. The treatment was tolerated well and no skin irritations occurred.