Background: Pseudoxanthoma elasticum(PXE) is a genetic multisystem disorder characterized by calcified dystrophic elastic fibres in skin, retina and arteries. Much of the earlier literature on pregnancy in PXE contained reports of severe complications, leading some healthcare providers to advise women with PXE against becoming pregnant and some women with PXE to avoid pregnancy. Objectives: To evaluate the obstetrical outcomes and the incidence of pregnancy complications in women with PXE and to determine if pregnancy is associated with an adverse effect on the course of the disease. Methods: Women with PXE (n=407) answered detailed questionnaires regarding reproductive history and pregnancy as well as the course of their disease. The frequency of reported pregnancy outcomes and complications was determined. Severity indices for the major clinical manifestations of PXE were developed and correlated with gravidity of affected women aged 40 years or over. Results: Among the 306 respondents with PXE who had ever been pregnant, there were 795 pregnancies. Of these, 83%ended in live births and 1%in stillbirth. The median birth weight was within the normal range and the incidence of low birth weight for gestation was low. Hypertension occurred in 10%of pregnancies, gastric bleeding and retinal complications in < 1%, and 12%of pregnancies were associated with worsening of skin manifestations. There was no effect of gravidity and clinical severity on cutaneous (P=0.07), ocular (P=0.59) or cardiac (P=0.42) manifestations of PXE in women aged 40+years, nor did ever having been pregnant adversely affect these clinical severity indices. Of the 101 women who had never been pregnant, 17%made the decision because they were advised against becoming pregnant by a healthcare professional and 11%did not become pregnant because they feared an adverse outcome either in their pregnancy or disease. Conclusions: PXE is not associated with markedly increased fetal loss or adverse reproductive outcomes. The incidence of gastric bleeding, although probably higher than in the unaffected population, is much lower than previously reported, and retinal complications are uncommon. Although a few pregnancies were associated with worsening of skin manifestations, there was no correlation of either gravidity or ever having been pregnant with ultimate severity of skin, ocular or cardiovascular manifestations. There is no basis for advisingwomenwith PXE to avoid becoming pregnant, and most pregnancies in PXE are uncomplicated. Background: Pseudoxanthoma elasticum (PXE) is a genetic multisystem disorder characterized by calcified dystrophic elastic fibers in skin, retina and arteries. Much of the earlier literature on pregnancy in PXE contained reports of severe complications, leading some healthcare providers to advise women with PXE against To pregnant and some women with PXE to avoid pregnancy. Objectives: To evaluate the obstetrical outcomes and the incidence of pregnancy complications in women with PXE and determine if the pregnancy is associated with an adverse effect on the course of the disease. Methods: Women with The frequency of reported pregnancy outcomes and complications was determined. The weight of reported for major clinical manifestations of PXE were developed and correlated with gravidity of Affected women aged 40 years or over. Results: Among the 306 responden ts with PXE who had ever been pregnant, there were 795 pregnancies. Of these, 83% ended in live births and 1% in stillbirth. The median birth weight was within the normal range and the incidence of Low birth weight for gestation was low. Hypertension occurred in 10% of pregnancies, gastric bleeding and retinal complications in <1%, and 12% of pregnancies were associated with worsening of skin manifestations. There was no effect of gravidity and clinical severity on cutaneous (P = 0.07), ocular ( Of the 101 women who had never been pregnant, 17% made the decision because (P = 0.42) manifestations of PXE in women aged 40 + years, nor did ever have been pregnant adversely affect these clinical severity indices. they were advised against becoming pregnant by a healthcare professional and 11% did not become pregnant because they feared an adverse outcome either in their pregnancy or disease. Conclusions: PXE is not associated with markedly increased fetal loss or adverse reproductive incidence. The incidence of gastric bleeding, although probably higher than in the unaffected population, is much lower than previously reported, and retinal complications are uncommon. Although a few pregnancies were associated with worsening of skin manifestations, there was no correlation of either gravidity or ever having been with ultimate severity of skin, ocular or cardiovascular manifestations. There is no basis for advising women with PXE to avoid becoming pregnant, and most pregnancies in PXE are uncomplicated.