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Pseudoxanthoma Elasticum
(PXE)

Medical Author: William C. Shiel Jr., MD, FACP, FACR

What is pseudoxanthoma elasticum?

The connective tissues are the structural portions of the body that essentially hold the body cells together. These tissues form a framework or matrix for the body. The connective tissues are composed of two major structural molecules, collagen and elastin. There are many different collagen protein types which vary in abundance depending on body area. Elastin is another protein which has the capability of stretching and returning to original length like a spring.

Pseudoxanthoma elasticum (PXE) is a rare disorder of degeneration of the elastic fibers with tiny areas of calcification in the skin, back of the eyes (retinae), and blood vessels. Interestingly, while elastin is the major component of ligaments (tissues which attach bone to bone), the ligaments are not apparently affected by PXE.



Next: How is pseudoxanthoma elasticum inherited? »

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