Estimates of the incidence of PXE range from 1 in 100,000 people to 1 in 25,000 people. However, the true incidence of PXE is not known in any population.

Vascular Disease

PXE frequently causes mineralization in the elastic layers of medium-sized arteries. This causes narrowing of blood vessels, so affected individuals may have decreased blood flow to the arms and legs. Decreased flow of blood to the arms and legs may mean that one's pulse can no longer be felt in the wrists or feet. This decreased flow of blood to the arms and legs may also cause cramping or pain in the legs or arms when walking or exercising. This cramping is called intermittent claudication.

Arterial narrowing and reduced blood flow can also cause angina (chest pain), heart attack, small strokes and intestinal angina (abdominal pain).

It is thought that hypertension (high blood pressure) is more common among people with PXE than in the general population, but no large study has been done to substantiate this. Hypertension should be aggressively treated to reduce the risk of heart attack and stroke.

It has been reported that mitral valve prolapse occurs more frequently in people with PXE than in the general population. These reports, however, have not been substantiated in large studies.

Individuals with PXE should have periodic visits to their physician for monitoring of blood pressure, cholesterol, and pulses in the arms and legs. A heart-healthy lifestyle is recommended, with low fat foods and plenty of exercise. Consistent exercise may decrease the effects of PXE on the blood vessels. Maintaining normal weight may also be beneficial. Smoking should be avoided.