Pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE) is an inherited disorder in which elastic fibers in three organ systems (the skin, retina of the eyes, and cardiovascular system) become slowly calcified.


This is a hereditary disorder of elastic tissue. Degenerating elastic tissue in the skin produces lesions which look like soft yellow papules. Elastic tissue in the eye and blood vessels is also involved, giving rise to visual impairment, raised blood pressure and haemorrhages.


An autosomal recessive disease that affects elastic tissues in cardiovascular tissues and skin. Abnormalities in the retina may result in the finding of angioid streaks on physical examination, and blindness. Arterial damage in other organs can result in ischemia. Skin lesions include yellow xanthomatoid patches and excess skin laxity.


A genetic condition affecting the connective or elastic tissue, characterized by early onset aging, deterioration of the skin, and degeneration of the arteries resulting in hemorrhages.


 


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