2002 Volume 112 Issue 11 Pages 1481-1486
Seven patients with diffuse cutaneous systemic sclerosis (dSSc) manifesting rapidly progressing skin sclerosis were treated with initial oral prednisolone of 20-30 mg/day for longer than 20 weeks. The dose of prednisolone was gradually decreased, and afterwards 5-10 mg/day was maintained. Four of these patients with severe skin sclerosis additionally received 200-300 mg/day d-penicillamine. Three patients with limited cutaneous SSc exhibiting mainly scleredema of the hands and forearms also took oral prednisolone. All patients but one with dSSc had disease durations of less than two years. All patients demonstrated marked improvement of skin sclerosis within 4-20 weeks and showed no relapse. In addition, two patients with longer disease duration and stable skin condition were treated with initial doses of 20 or 40 mg/day prednisolone because of mononeuritis and interstitial pneumonia, respectively, and exhibited improved skin sclerosis. Furthermore, one patient given 1,000 mg/day methylprednisolone intravenously for two days due to toxic epidermal necrolysis (TEN) presented significant improvement in skin sclerosis. Adverse reactions to corticosteroid included steroid diabetes and osteonecrosis of the femoral head in one patient each and that to d-penicillamine included TEN in one patient. Low- or moderate-dose oral prednisolone seems to be effective for skin sclerosis of SSc, especially of early SSc.