Pseudoxanthoma Elasticum (PXE)

Pseudoxanthoma Elasticum (PXE) is a systemic disease that can affect the eye.

PXE is not an eye disease, so to speak -- however, its effects can be seen in the eye.  PXE can cause a weakening, or cracking, of one of the important layers in the retina -- Bruch's Membrane.  These "cracks" are called Angioid Streaks.  When angioid streaks occur in Bruch's membrane, complications such as choroidal neovascularization can arise.

Choroidal neovascular membrane (CNVM) is a development of new, abnormal blood vessels that sprout up into the layers of the retina.  These abnormal vessels originate in the vascular layer, below the retina.  CNVM blood vessels leak, by nature, and cause swelling, thickening and even bleeding within the retina.  These affects cause the vision to weaken or blur.

Although angioid streaks can not be treated, CNVM can be treated a multitude of ways, including: Macugen, Lucentis, Avastin, Kenalog, Photodynamic laser and traditional laser surgery.

Commonly, a fluorescein angiogram, a dye study of the retina, will be used to locate and diagnose leaking CNVM.

Self monitoring with an Amsler grid is recommended for patients with angioid streaks or PXE.  Regular dilated eye exams with an ophthalmologist is suggested as well.

More information on PXE can be found at http://www.pxe.org