Stargardt disease. A, Fundus photograph shows diffuse retinal flecks. B, FAF imaging reveals increased autofluorescence corresponding to the retinal flecks (red asterisk) and decreased autofluorescence corresponding to areas of RPE atrophy (green asterisk). C, SD-OCT image shows hyperreflectivity at the level of the RPE (corresponding histologically with enlarged RPE cells with increased lipofuscin content) (arrows), markedly thinned retina in the foveal region, and focal attenuation or loss of the photoreceptor cell layer in areas corresponding to RPE atrophy (area between arrowheads). D, Photomicrograph of a PAS-stained section demonstrates hypertrophic RPE cells (arrowheads) with numerous PAS-positive cytoplasmic granules containing lipofuscin (asterisks). This histologic finding corresponds to the retinal flecks seen clinically. E, In advanced stages of Stargardt disease, geographic RPE atrophy with loss of the photoreceptor cell layer (between arrows) may be noted.