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The name pattern dystrophy of the retinal pigment epithelium (RPE) groups together a series of macular conditions previously named with a variety of descriptive terms and eponyms. Some examples include butterfly dystrophy, Sjögren's reticular dystrophy, fundus pulverulentus and adult vitelliform dystrophy or degeneration (adult Best's disease).
These conditions tend to share the following features:
The fact that different types of pattern dystrophy can be seen within the same family lends support to the notion of a single disease process. Also the pattern of the macular pigmentation may change with the passage of time. The Sjögren's reticular type can be inherited as an autosomal recessive trait. In the autosomal dominant form of the disease, the peripherin/RDS gene, which is located on chromosome 6, is thought to be defective.
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