Figure 3. Color (left) and autofluorescence (right) fundus photographs from the right eye of patient II-6, recorded at the age of 35 years. Fluorescence was absent in a hyperpigmented central zone of outer retinal atrophy, whereas an irregular gray to white surrounding zone showed confluent hyperautofluorescence. The general level of fundus autofluorescence outside the macula appeared to be normal.