Pregnancy in women with pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE) is characterized by degeneration of elastic tissue. Clinically, its effects are most readily noted in the skin, retina, blood vessels, and myocardium. Several hundred patients with this condition, signs and symptoms of which usually appear during the early portion of the reproductive years, have been reported. Fertility is unaffected. Despite this, only five articles describing the outcome of pregnancies in seven women with PXE have been recorded, none in the obstetric literature. This is particularly odd considering that five of the seven women suffered serious gastrointestinal hemorrhage during one or more of their pregnancies. One woman suffered congestive heart failure and cardiac arrhythmia during pregnancy, and a fatal attack of cardiac arrhythmia 2 months postpartum. The five articles are reviewed, basic information regarding PXE is given, a case history is recorded, and the possibility that pregnancy favors the development of these complications in patients with PXE is discussed.