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September 1963

Pseudoxanthoma Elasticum With Gastric Hemorrhage

Author Affiliations


Clinical instructor in medicine, University of Rochester School of Medicine and Dentistry, and assistant physician, Strong Memorial Hospital (Dr. Flatley); medical officer, US Naval School of Aviation Medicine, Pensacola, Fla, formerly intern in medicine, Strong Memorial Hospital (Dr. Atwell); clinical instructor in surgery, University of Rochester School of Medicine and Dentistry, and assistant surgeon, Strong Memorial Hospital (Dr. McEvoy).; From the Department of Medicine of the University of Rochester School of Medicine and Dentistry and Strong Memorial Hospital.

Arch Intern Med. 1963;112(3):352-356. doi:10.1001/archinte.1963.03860030106010

Pseudoxanthoma elasticum (PXE) is a rare, heritable, systemic disorder of elastic tissue and is primarily manifested by abnormalities of the skin, ocular fundus, and cardiovascular system.1 Gastrointestinal hemorrhage, frequently recurrent, has been reported in association with PXE and may constitute the major hazard to life. Although peptic ulcers, esophageal varices, and ulcerative colitis have been present in some cases, the source of bleeding is most commonly undetermined.2-15

Involvement of the visceral vessels with elastic tissue degeneration has been postulated as the most likely cause of unexplained bleeding. However, in only five previously reported cases has histologic examination of visceral vessels been performed to substantiate this theory.16-19

This report describes the clinical, radiological, and pathological findings in a 37-year-old white male with previously undiagnosed PXE who underwent subtotal gastrectomy for persistent gastric hemorrhage.

Report of Case  A 37-year-old, single, white male clerk was admitted to Strong Memorial Hospital