The pattern dystrophies

The pattern dystrophies form a clinically and genetically heterogeneous group of retinal phenotypes including adult-onset foveomacular vitelliform dystrophy (AFVD), butterfly-shaped pigment dystrophy (BPD), reticular dystrophy of the retinal pigment epithelium, pseudo-Stargardt pattern dystrophy (multifocal pattern dystrophy simulating Stargardt disease/fundus flavimaculatus), and fundus pulverulentus. The pattern dystrophies constitute a group of retinal disorders characterized by a variety of deposits of yellow, orange, or gray pigment, predominantly in the macular area. The age at onset in pattern dystrophies is highly variable, but patients tend to remain asymptomatic until the 5th decade or may even remain asymptomatic throughout life. The course of pattern dystrophies is often benign, although severe vision loss occurs in up to 50 % of the affected individuals after the age of 70, as a result of chorioretinal atrophy and/or the development of choroidal neovascularization.