PXE results in a variety of signs and symptoms. The number, type, and severity of signs of PXE vary from person to person. Certain effects of PXE can cause serious medical problems while others have less impact. The effects of PXE may include: skin changes; changes in the retina of the eye that may result in significant loss of central vision; changes in the cardiovascular system that may involve calcification of arteries and decreased blood flow in the arms and legs or even the heart and brain; changes in the gastrointestinal system that may lead to bleeding in the stomach or intestines.
At present, there is no way to predict the progression of the disorder for a particular individual. Some people have no skin lesions; others have no vision loss. Many people do not experience gastrointestinal complications or cardiovascular difficulties. And a few have no manifestations of PXE except for a positive skin biopsy or angioid streaks. The effects of PXE and its rate of progression seem to have no discernable pattern. Thus there is great variability in how PXE affects each person.