Moreover, altered expression of DCN has been detected in a number of human diseases with skin phenotypes, including decreased DCN in fibroblasts in patients with neonatal Marfan syndrome (Raghunath et al., 1993), increased DCN in fibroblasts from patients with localized scleroderma (Izumi et al., 1995), and increased DCN in fibroblasts from patients with systemic sclerosis (Westergren-Thorsson et al., 1996). Here, DCN is linked to systemic sclerosis.