Occasionally there may be lesions around the navel, inside the lips, or in the lining of the vagina or rectum. The skin changes in PXE vary from person to person. In some individuals there will be very little discernible change. In others the plaques coalesce. Eventually the skin may become lax and folded.
Some individuals choose plastic surgery which appears effective.
The only other problem that skin changes cause is psychosocial, that is the person with lax and folded skin may be uncomfortable with its appearance. It is called this because the membrane behind the eye begins to resemble the skin of an orange. This is probably caused by calcification of Bruch's membrane. If the calcifications continue, the membrane may develop cracks, which are called angioid streaks. Angioid streaks are the most diagnostic feature of PXE in the eye.