Pseudoinflammatory macular dystrophy

We studied a family with a dominantly inherited macular dystrophy resembling Sorsby's pseudoinflammatory dystrophy. Retinal pigment epithelial atrophy and varying degrees of pigment epithelial metaplasia were prominent fundus features in this pedigree. However, findings on electro-oculography were abnormal, unlike previous findings in patients with Sorsby's dystrophy. Histopathologic study of an eye from one patient demonstrated widespread atrophy of the retina, retinal pigment epithelium, and choroid. Although the pseudoinflammatory fundus appearance is common to several macular dystrophies and some eyes with end-stage, age-related macular degeneration, abnormal electro-oculograms and a dominant inheritance pattern distinguish the dystrophy in the present pedigree from other dystrophies and age-related macular degeneration.