Pattern dystrophy of the pigment epithelium.
Three generations of a family showed alterations in the retinal pigment epithelium along with mild visual disturbance. Younger members had granular pigment dispersed beneath the fovea while older members showed a reticular pigment pattern. The electro-oculogram was subnormal in most affected individuals, but visual fields, color vision, dark adaptation, and the electroretinogram were normal. The pigmentary changes in these patients are similar to those seen in other pigment epithelial dystrophies such as reticular dystrophy and butterfly-shaped dystrophy. Until these disorders can be separated pathophysiologically, we suggest the definition of a more inclusive clinical term, "pattern dystrophies of the pigment epithelium.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003623:Dark Adaptation; D004594:Electrophysiology; D004596:Electroretinography; D005260:Female; D006801:Humans; D008268:Macular Degeneration / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010857:Pigment Epithelium of Eye; D012162:Retinal Degeneration / Q000235:genetics*; D014794:Visual Fields