Reticular-like pigmentary patterns in pseudoxanthoma elasticum.

Of 14 consecutive patients with pseudoxanthoma elasticum (PXE), 9 (64%) had a spectrum of peculiar, sometimes reticular, pigmentary changes in the retina. Ten of 18 (56%) eyes had a random scattering of small, round pigment dots throughout the macula and around the optic nerve, sometimes extending to the equator. In four of 18 (22%) eyes, the pigment clumps appeared in lines, resembling a string of pearls. These strings occasionally radiated from the macula in a spoke-like fashion. In 4 of the 18 (22%) eyes, the pigment clumping occurred in a fishnet, or truly reticular, pattern. Of the 18 eyes in these 9 patients, 12 (67%) had subretinal neovascularization. Of the ten eyes in the five patients who did not show this pigmentary change, two (20%) had subretinal neovascularization.