Pseudoxanthoma elasticum natural history, complications and prognosis

The ocular involvement including retinal hemorrhages can progressively lead to loss of central vision, sparing the peripheral vision. The involvement of the elastic media and intima of the arteries can lead to claudication, hypertension, angina pectoris, myocardial infarction, and gastrointestinal or cerebral hemorrhage which could be fatal, although relatively uncommon. The prognosis of PXE largely depends on the extracutaneous organ manifestations. The occurence of myocardial infarction, cerebral or GI hemorrhage may have fatal consequences. Spontaneous resolution of skin changes has been reported, but is exceedingly rare.^[1]

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