Cutaneous sclerosis, assessed yearly by a semiquantitative skin scoring technique (sum of 10 body areas, each scored on a scale of 0–3 for tethering, by clinical palpation), and outcome at 10 years were evaluated in 90 systemic sclerosis patients enrolled in a 3-year, prospective drug trial. The inverse relationship of survival to the skin score at study entry (increasing skin score associated with decreasing survival) (P < 0.003) was more significant than was the relationship found by using classification schemata that rely only on distribution of cutaneous sclerosis. Six-year survival was 40% in patients with entry skin scores ≥15 and 73% when skin scores were <15 (P < 0.005). An entry skin score ≥15 was associated with a high risk of early fatal renal and cardiac complications.