GASTROINTESTINAL MANIFESTATIONS OF PXE

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by Drs. Aaron Walfish & Allen Sapadin
(from Volume 6, Issue 3, 1998)

The gastrointestinal (GI) manifestations of PXE are thought to result from the calcification of elastic tissue in the gastrointestinal arterial wall which can lead to thinning, aneurysm formation, and rupture. Hemorrhage, specifically in the stomach, is the most common GI presentation in patients with PXE, although jejunal and rectal bleeding have also been described. Although most cases of GI hemorrhage occur between the ages of 20 and 30 years, bleeding has also been seen in pediatric patients under age 10.

GI bleeding develops in approximately 13% of patients with PXE. There is an increased incidence during pregnancy and it frequently occurs before the eye and skin changes have fully emerged. Patients are predisposed to recurrent episodes of acute GI hemorrhage due to continued elastic fiber deterioration in arterial walls and possibly because of failure of vascular constriction. Frequently the bleeding is not serious and stops spontaneously so that the problem can go undetected. Occasionally when severe stomach bleeding does occur endoscopic or even surgical intervention may become necessary.

Although severe bleeding is rare in pregnant patients, it is still important for PXE patients to be aware of this risk. The obstetrician who is following the pregnant patient should periodically perform stool examinations for the detection of occult blood.