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.