Multifocal Best's disease and sickle cell trait

Ophthalmoscopy performed during a routine eye checkup on a 22-year-old black man revealed multiple intraretinal, cyst-like lesions scattered throughout the macular and extramacular areas in both eyes. Based on clinical findings, morphologic appearance, and an abnormal electro--oculogram, the diagnosis of multifocal Best's macular dystrophy was made. Hemoglobin electrophoresis showed an AS pattern confirming the presence of sickle cell trait. This is the first case of the coexistence of these two genetically transmitted diseases, Best's disease and sickle cell trait.