BEST1 and Behcet disease: Since then, over 250 distinct BEST1 mutations have been linked to BD, and later to a lesser extent also to three other distinct retinal pathologies (https://databases.lovd.nl/shared/genes/BEST1, https://www.ncbi.nlm.nih.gov/clinvar), namely the autosomal dominant adult-onset vitelliform macular dystrophy (AVMD) (Krämer et al., 2000), the autosomal dominant vitreoretinal choroidopathy (ADVIRC) (Yardley et al., 2004) and the autosomal recessive bestrophinopathy (ARB) (Burgess et al., 2008).