Fuchs' heterochromic cyclitis associated with retinitis pigmentosa: a family study.

To determine the hereditary, clinical histopathological aspects of the association between Fuchs' heterochromic cyclitis (FHC) and retinitis pigmentosa (RP), the family of a patient with FHC and bilateral RP was studied genealogically, ophthalmologically and immunogenetically. The oldest brother and the youngest sister of the proband had bilateral RP and glaucoma which in the brother lead to enucleation of an eye which was studied histologically. The proband, his brother with RP, and 2 of their healthy siblings were homozygous for the haplotype A3, B7, w6. The parents of the siblings were healthy, and the pedigree showed much parental consanguinity and indicated autosomal recessive inheritance of RP.