Atypical drusen in pseudoxanthoma elasticum

Forty-three patients with pseudoxanthoma elasticum were evaluated ophthalmologically. The fundus appearance was characterized by angioid streaks and mottling of the retinal pigment epithelium. Rock-like atypical drusen were present in the posterior pole or peripheral retina in 75% of the patients examined and were an integral part of this fundus picture. These drusen changed little with time and were seen in early stages of pseudoxanthoma elasticum. The histopathology of these drusen is unknown.