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Cutaneous and ophthalmological features of PXE. Skin lesions include yellowish papular lesions in neck and flexural areas (a–c), often coalescing into larger plaques (b). In fundo, angioid streaks (d, arrowed) can be observed, as well as a mottled aspect of the fundus called peau d'orange (e, asterisk). Following neovascularisation, subretinal haemorrhage and vision loss can occur (f).
