Central areolar pigment epithelial (cape) dystrophy.
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.
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D008268:Macular Degeneration / Q000235:genetics* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010857:Pigment Epithelium of Eye / Q000473:pathology; D012162:Retinal Degeneration / Q000235:genetics*; D014792:Visual Acuity