 | Joseph P. Galichia, MD is the founder and Medical Director of the Galichia Medical Group, PA. He is an internationally recognized pioneer in the field of interventional cardiology. In the 70's, Dr. Galichia studied in Zurich, Switzerland with the inventors of the balloon angioplasty technique for treating heart disease. As a result of his experience, he was able to bring this historic technique back to the United States where he was one of the first physicians to perform coronary angioplasty here. A noted medical spokesman, he appears on a weekly Newstalk segment on KWCH Channel 12 and has a weekly syndicated radio talk show on KNSS 1330 AM every Saturday live from 11:00 am to noon. Dr. Galichia may be contacted by sending an e-mail to service@galichia.com |
Health & Medicine
2008-02-01 10:12:00
What is PXE?
What exactly is PXE (Pseudoxanthoma Elasticum)?
PXE is an inherited systemic disease characterized by changes in the elastic tissue of the skin. This disorder affects mainly the skin, eyes, heart and gastrointestinal system. The skin changes are distributed on the flexor regions of the extremities, in the folds in the skin, at the sides of the neck, under the arm, and behind the knee. Yellowish pebbly bumps give the skin a plucked chicken appearance. PXE appears to be inherited as a dominant gene and affects twice as many females as males. A diagnosis of PXE is determined through biopsy.
The basic PXE disorder seems to be characterized by increased amounts of elastic tissue, and a tissue that has a tendency to become calcified. No one knows why the elastic tissue forms as this continues to be a subject of intense research. In the United States, PXE is reported as being prevalent at a level of one in 160,000 births. Many of these people live a normal lifetime but weakening of the blood vessels in the brain, and upper GI tract hemorrhaging are serious complications of PXE due to the degeneration of the elastic tissue of blood vessels and arteries of the gastric wall. Most patients are diagnosed in their thirties or forties. Patients with PXE have progressive involvement of the skin, eyes and other organ systems with extremely stretchable skin being a common characteristic.
About 87% of these patients have changes in their retina which is a result of this disease. Loss of vision may occur around the age of 40 and this can tend to be progressive.
To date, there is no real treatment for this disease. Patients with PXE should have regular eye exams and see their physician regularly to monitor blood pressure and any vascular changes as hypertension and vascular disease are fairly common in patients with PXE.
