The obstetric and gynaecological implications of pseudoxanthoma elasticum

The obstetric aspects of 54 pregnancies in 20 women with pseudoxanthoma elasticum (PXE) have been reviewed. Forty live births were delivered vaginally, three of them were preterm. Twelve pregnancies miscarried at between 6 and 14 weeks gestation and two were terminated in the 8th week for psychiatric reasons. Fetal losses in two instances were attributed to cervical incompetence. One baby died at 4 days of age from a complex congenital cardiac anomaly. No abnormal maternal genital or gastrointestinal bleeding was recorded. Hypertension complicated seven pregnancies. Abdominal striae developed in all patients, the severity of which correlated with weight gain and the number of pregnancies. All the women had normal gynaecological histories, none had excessive menstrual bleeding or increased episodes of menorrhagia. The eye, blood vessels and other organ systems involved in PXE were unaffected by pregnancy. Apart from the increased risk of first trimester miscarriage and the cosmetic deterioration of the abdominal skin, there are few serious complications in pregnancy in patients with PXE.