Central areolar choroidal dystrophy

Inheritance is autosomal dominant Presentation is in the 20's to 30's with gradual impairment of central vision. Signs in chronological order: 1) Non-specific foveal granularity. 2) Circumscribed RPE atrophy and loss of the choriocapillaris at the macula. 3) Slowly expanding geographic atrophy with prominence of large choroidal vessels . Prognosis is poor with severe visual loss occurring by the 50's to 60's. So if you see a younger patient with macular pigment changes orgeographic atrophy, consider this diagnosis.