Pseudoxanthoma elasticum
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Reviewed August 2007
What is pseudoxanthoma elasticum?
Pseudoxanthoma elasticum is a progressive disorder that is characterized by accumulated deposits of calcium (calcification) and other minerals in elastic fibers, which are a component of connective tissue. Connective tissue provides strength and flexibility to structures throughout the body.
Calcification can affect elastic fibers in the skin, eyes, and blood vessels, and less frequently in other areas such as the digestive tract. As a result, people with pseudoxanthoma elasticum may have yellowish bumps called papules on their necks, underarms, and elsewhere on the skin. They also may have changes in the eye called angioid streaks, which occur in the light-sensitive layer of cells at the back of the eye (the retina). Angioid streaks can lead to bleeding and scarring of the retina, which can cause vision loss.
Calcification of elastic fibers in the arteries can cause decreased blood flow to the arms and legs, resulting in cramping or pain with exercise (claudication). If the coronary arteries in the heart are affected, calcification can result in chest pain on exertion (angina) or rarely, a heart attack. Bleeding from blood vessels in the digestive tract may also occur.
Calcification can affect elastic fibers in the skin, eyes, and blood vessels, and less frequently in other areas such as the digestive tract. As a result, people with pseudoxanthoma elasticum may have yellowish bumps called papules on their necks, underarms, and elsewhere on the skin. They also may have changes in the eye called angioid streaks, which occur in the light-sensitive layer of cells at the back of the eye (the retina). Angioid streaks can lead to bleeding and scarring of the retina, which can cause vision loss.
Calcification of elastic fibers in the arteries can cause decreased blood flow to the arms and legs, resulting in cramping or pain with exercise (claudication). If the coronary arteries in the heart are affected, calcification can result in chest pain on exertion (angina) or rarely, a heart attack. Bleeding from blood vessels in the digestive tract may also occur.
How common is pseudoxanthoma elasticum?
The estimated prevalence of pseudoxanthoma elasticum ranges from 1 in 25,000 to 1 in 100,000 individuals. The true prevalence is probably higher because mildly affected people are often not diagnosed. For reasons that are unknown, this disorder is diagnosed twice as frequently in females as in males.
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