A young female with subretinal thread-like structures & neck
A 21-year-old female was found to have bilateral multiple irregularly branching interconnected subretinal thread-like black lines radiating from the optic disc. Visual acuity was 20/20 in either eye. The fundus fluorescein angiogram (FFA) images (as attached).

The images revealed angioid streaks around the optic disc and typical peau d'orange appearance temporal to the fovea, window defects corresponding to the angioid streaks. The fovea was healthy, and there was no evidence of choroidal neovascular membrane (CNVM).

This female had asymptomatic coalescing yellowish plaques on the posterolateral side of her neck, “plucked chicken” appearance.

Biopsy followed by hematoxylin-eosin and Verhoeff Van Gieson staining showed irregularly clumped calcified elastic fibers in reticular dermis confirming PXE.

The patient was explained to follow-up with the ophthalmologist regularly and to report urgently in case of metamorphopsia or vision loss which may denote the development of a CNVM.

Clinical pearls:-
Pseudoxanthoma elasticum (PXE), Ehlers Danlos syndrome, Paget's disease, sickle cell anemia, acromegaly, diabetes mellitus, and other systemic diseases should be ruled out in patients with angioid streaks.

Source: https://pxmd.co/3WcdK
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