Figure 1: Colour fundus montage of the right (A) and left eye (B) shows a greyish opacification of the peripapillary retina; with more details of the posterior pole (C, D). FAF imaging reveals a speckled hyper-autofluorescence denoting an active disease phase and a speckled hypo-autofluorescence indicating RPE atrophy (E, F). OCT reveals a zonal loss of the outer retinal layers with a disruption of the photoreceptors (arrows; G, H).