The term pATTERN dystrophy was suggested by the author¹² and Hsieh et al. in 1977⁷ to describe a group of related dystrophies of the retinal pigment epithelium (RPF) that are characterized by granular or reticular pigmentation patterns and a relatively benign clinical course. Earlier literature had described a variety of RPE dystrophies with unusual pigmentary patterns such as reticular dystrophy,¹⁸ butterfly dystrophy,⁵ fundus pulverulentus,¹⁹ dystrophia macroreticularis,¹⁵ and others. Some families with dominant pattern dystrophy, often with a butterfly pattern, have now been found to have mutations of the peripherin/RDS gene (a gene that can also cause retinitis pigmentosa).^9,16,17 And a pattern dystrophy has been described in families with a mitochondrial DNA mutation that also causes maternal diabetes and deafness.^2,14 However, many families lack any known mutation,^13,22 and inheritance can be recessive as well as dominant. Thus, the designation “pattern dystrophy” provides a way of describing and categorizing a variety of genetic disorders that behave in a similar fashion with respect to RPE involvement and limited functional changes.¹¹