Best vitelliform macular dystrophy (BVMD) is a genetic macular dystrophy characterized by loss of central visual acuity, metamorphopsia and a decrease in the Arden ratio secondary to an egg yolk-like lesion located in the foveal or parafoveal region. The disease is vitelliform macular dystrophy 2 (MONDO_0007931). Also known as: BEST1 retinopathy, BMD, BVMD, Best Vitelliform Macular Dystrophy, Best disease, Best macular dystrophy, early-onset vitelliform macular dystrophy, juvenile-onset vitelliform macular dystrophy.