Figure 2.

Progression of angioid streaks and peau d’orange in patients with pseudoxanthoma elasticum (PXE). A–C, Fundus photographs of the right eye of a female patient at (A) 9, (B) 21, and (C) 28 years of age. The first fundus photograph (A) reveals only peau d’orange, which progressed together with the (B) appearance and (C) growth of angioid streaks. D–I, Fundus photographs (D–F) and corresponding 30° near-infrared reflectance (NIR) imaging (G-I) from a female patient at 14 years of age with follow-up imaging after 37 months (at 17 years of age) and 56 months (at 19 years of age). The white arrowheads indicate the development of a new angioid streak in the macula (H), which grows further in less than 2 years (I). Progression of the central border of peau d’orange is visible on 30° NIR imaging and is indicated with dashed lines (G, I). J–M, Growth of or appearance of new angioid streaks were assessed with 55° NIR imaging in two patients. J, K, Lengthening of an angioid streak (K) with a follow-up interval of 56 months after the first visit at 27 years of age (J). L, M, Occurrence of new angioid streaks presenting as a new branch nasal of the optic nerve visible in a patient 47 years of age (L) at baseline with (M) an interval of 63 months. The black arrowheads in (F, J) indicate islands of decreased reflectivity in NIR imaging, so-called temporal sparing.