Eleven family members over four generations have had granulomatous disease of the skin, eyes, and joints. Ten have had arthritis; two had skin, eye, and joint involvement; one had skin and joint disease, and one had iritis only. The disease is transmitted as an autosomal dominant trait and is not associated with HLA-B27. The disease resembles sarcoidosis in some ways but not in others, and is probably a new syndrome. The major long-term problems are iritis and joint contractures.