Doyne's honeycomb retinal degeneration. Clinical and genetic features.

IN 1899 Doyne described a fundus condition which he had observed in four sisters. The ophthalmoscopic appearance was of closely-grouped white spots involving the disc-macular area in a pattern he termed "Honeycomb". He believed the lesions were probably due to an exudate in the choroid, and therefore called the condition honeycomb choroiditis (Doyne, 1910), although it was shown shortly afterwards by Treacher Collins (1913) that the white spots were nodular thickenings of Bruch's membrane and that the choroid was essentially normal. The number of white spots increased with ageing until late in life when they became confluent to produce a homogeneous atrophic area enclosing the optic disc and macular areas. Doyne recognized that the lesion was inherited and even drew up a pedigree (unpublished) of three families in which the condition had been found. He was unable to comment on the type of heredity as at that time mendelian principles were only just being rediscovered. Later, some of Doyne's families were followed up and reported on, notably by Tree (1937) and Franceschetti, Franqois, and Babel (1963). The condition has been found to exist elsewhere in Britain (Foster, 1932) and in other parts of the world (Alper and Alfano, 1953; Pajtas, 1950; Fuchs, 1956). A genetical survey of this condition was recently undertaken in the areas from which Doyne drew his cases and various aspects of this survey are described in a separate paper (Pearce, 1967). The present communication outlines the main genetic findings and describes, among those found affected, the considerable variation noticed both in the ophthalmoscopic appearance and in the degree of visual disturbance. The value of these features in assessing prognosis is also discussed.