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 less than 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 greater than or equal to 15 and 73% when skin scores were less than 15 (P less than 0.005). An entry skin score greater than or equal to 15 was associated with a high risk of early fatal renal and cardiac complications.