Nummular pigmentary deposition alone is not specific to ESCS and has been described in other retinal dystrophies such as Bardet-Biedl syndrome,70CRB1-associated early-onset severe retinal dystrophy,71 retinitis pigmentosa with preserved para-arteriolar RPE (RP12, associated with CRB1),72 and thioridazine retinopathy.73 This evidence concerns the gene CRB1 and Bardet-Biedl syndrome.