RP1L1 and cone dystrophy: An environmental agent or pathogen may create an autoimmune response against short wavelength (S-cone) photoreceptors modulated by a pathogenic variant in cone dystrophy genes like RP1L1. In case 2, we cannot rule out the contribution of hydroxychloroquine toxicity to her phenotype, although photoreceptor damage to the arcades would be unusual without prominent RPE changes.