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Currently, diagnosis of PXE relies on clinical examination for characteristic skin lesions and angioid streaks or von Kossa staining of a biopsy of skin lesions looking for calcification of dystrophic dermal elastic fibres.
DISCUSSION: Angioid streaks are irregular, radiating, jagged, tapering lines that extend from the peripapillary area into the peripheral fundus that may occur in isolation or as the ocular manifestation of a systemic disease [1].
3) Risk of cardiovascular disease may correlate with the presence of angioid streaks of the fundus--irregular, reddish-brown, or gray lines radiating from the optic disc.
Angioid streaks are often associated with SCD and are thought to be the result of vessel occlusions in the choroidal circulation and associated breaks in Bruch's membrane (see Figure 4).
1) The most important fundus finding is angioid streaks.
The changes in Bruch's membrane give rise to angioid streaks, and rupture of the retinal vessels to hemorrhages and choroiditis.
The most important finding of PXE in the fundus is angioid streaks.