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April 1979

Autosomal Dominant Dystrophy of the Retinal Pigment Epithelium

Author Affiliations

From the Wilmer Ophthalmological Institute and Department of Pathology (Drs O'Donnell and Green), Divisions of Cardiology and Clinical Pharmacology, Department of Medicine (Dr Reid), The Johns Hopkins Medical Institutions, Baltimore, and the Department of Ophthalmology, University of California Medical Center and St Mary's Hospital and Medical Center (Dr Schatz), San Francisco.

Arch Ophthalmol. 1979;97(4):680-683. doi:10.1001/archopht.1979.01020010336010

• This study describes a salt-and-pepper fundus appearance in nine relatives in three generations of a single white kindred. Two of these patients also had subretinal patterned pigment figures by ophthalmoscopy. In another, fluorescein angiography showed a reticular pattern that was not apparent by ophthalmoscopy. This dystrophy was functionally benign, although the electro-oculogram ratio was abnormal in affected patients. This pigmentary dystrophy and fundus pulverulentus, reticular dystrophy of Benedikt and Werner, and pattern dystrophy of Marmor and Byers are indistinguishable. When patterned pigment figures are absent, this autosomal-dominant pigmentary dystrophy of the retinal pigment epithelium can be confused with other disorders such as congenital rubella retinopathy and drug toxicity.

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