Diffuse and limited cutaneous systemic scleroderma

Juvenile systemic scleroderma (jSSc) is a rare disease. Based on the first large data collection on this patient group, the disease course was demonst rated to differ from that in adults. The concept of persistence of maternal cells in patients with SSc remains pathogenetically fascinating, as does t he resemblance with graft-versus-host-disease. In View of new therapeutic o ptions, controlled trials have not established a gold standard for treatmen t, but autologous bone marrow transplantation may be considered a rescue th erapy for selected patients, Palliative therapies have also improved marked ly in recent years. The first controlled trials for patients with jSSe are being proposed. (C) 2000 Lippincott Williams & Wilkins, Inc.