Cardiovascular involvement often develops after the skin and eye lesions and then most commonly involves the peripheral arteries in the legs and arms. It usually begins suddenly with few warning signs and may be severe enough to require hospitalization and blood transfusions. Research in these areas has been woefully inadequate, due in large measure to the rarity of the disorder and the consequent difficulty in obtaining funding for basic research. The abnormal gene for PXE is passed from generation to generation usually without the knowledge of the individuals who pass and receive it. Most cases are inherited in a so-called autosomal recessive pattern.
Some families show an autosomal dominant inheritance pattern.
There are also no adverse effects on the newborn infants. Studies have shown, however, that women who have had several pregnancies do have more adverse effects from their PXE later in life compared to those who have had fewer or no pregnancies. As with any hereditary condition, a cure is not possible at this time. The chromosome carrying the PXE gene has been recently discovered. Once the specific gene on the chromosome has been found, the possibility exists for a treatment aimed at a missing or defective gene product.