This patient with pseudoxanthoma elasticum (PXE) has classic extensive angioid streaks with peaux d’orange (orange skin). The orange skin appearance is caused by the calcified Bruch’s membrane giving a yellow mottled appearance that contrasts with the normal orange RPE and choroidal pigmentation. The green channel (532 nm) Optos images best show these findings in the RPE/Bruch’s membrane.
Learning Points:
Although angioid streaks can be seen in other disorders, including age-related and sickle-cell disease, the findings in our patient are pathognomonic for PXE.
These patients are at significant risk for macular neovascularization (MNV) and need to be followed and treated as needed with anti-VEGF agents, as in patients with AMD. Fortunately our patient has no MNV with normal macular OCTs (not shown).
