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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2025.

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Figure 4.

Figure 4.

Right eye of a male age 72 years with molecularly confirmed Best disease

A. Macula shows loss of photoreceptor cells and an attenuated outer plexiform layer (OPL).

B & C. Fovea shows deposits of PAS-positive material and pigment granules (arrows) without underlying RPE cells.

D. Hyperpigmentation of the RPE (red arrows) is seen in the perifovea.

Image courtesy of C-C Chan, National Eye Institute, NIH, retired

From: Bestrophinopathies

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