Central areolar pigment epithelial (cape) dystrophy.

Abstract A family with a unique hereditary macular dystrophy is presented. The disorder is transmitted as an autosomal dominant trait having high penetrance and variable expression. The characteristics of this dystrophy are: childhood onset; nonprogressive areolar depigmentation of the central macula; and with the exception of one eye with a macular hemorrhage, normal vision acuity and retinal-function studies.