Histopathologic features of adult-onset foveomacular pigment epithelial dystrophy

G. J. Jaffe and H. Schatz
Department of Ophthalmology, University of California, San Francisco.

We studied the clinical and histopathologic characteristics of the eyes obtained after death from a patient with adult-onset foveomacular pigment epithelial dystrophy. The pigmentation seen in the central fovea corresponded histologically to a hyperplastic clump of retinal pigment epithelium. The pale yellow rim surrounding the central pigmentation corresponded histologically to dense periodic acid-Schiff-positive material underlying thinned, atrophic retinal pigment epithelium. Fluorescence microscopy demonstrated homogeneous autofluorescence in the retinal pigment epithelium that was similar in intensity to that of an age-matched control. The results of this clinicopathologic study suggest that in adult-onset foveomacular pigment epithelial dystrophy, an alteration of macular retinal pigment epithelium causes an accumulation of abnormal subretinal pigment epithelial material, photoreceptor degeneration, and serous retinal detachment.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Molecular Physiology of Bestrophins: Multifunctional Membrane Proteins Linked to Best Disease and Other Retinopathies
Hartzell et al.
Physiol. Rev. 2008;88:639-672.
ABSTRACT | FULL TEXT  

Chloride Channel Activity of Bestrophin Mutants Associated with Mild or Late-Onset Macular Degeneration
Yu et al.
IOVS 2007;48:4694-4705.
ABSTRACT | FULL TEXT  

Morphological and functional analyses of adult onset vitelliform macular dystrophy
Saito et al.
Br. J. Ophthalmol. 2003;87:758-762.
ABSTRACT | FULL TEXT