Ménétrier disease is also called hypoproteinemic hypertrophic gastropathy.
Associated Ménétrier disease associated with enlarged rugae, forming giant folds in the lining of the stomach.
Rugae enlarge because of an overgrowth of surface mucous cells of the stomach.
Rugae release protein-containing mucus in a normal stomach and with enlarged rugae too much mucus is released.
This causes a leakage of protein from the blood into the stomach resulting in hypoproteinemia.
Ménétrier disease also causes a decrease in stomach acid resulting from a reduction in acid-producing parietal cells.
Patients complain of severe stomach pain, nausea, vomiting, and other symptoms.
Associated with a higher risk of developing stomach cancer.
Other conditions that can cause enlarged rugae include: Zollinger-Ellison syndrome, syphils, cytomegalovirus, histoplasmosis, linitis plastica, and gastric lymphoma.
When associated with CMV, usually in childhood.
Thought to be an acquired disorder with no known genetic component.
Patients have an abnormally high amounts of a protein called transforming growth factor-alpha (TGF-α).
More common in men, usually appearing between the ages of 30 and 60.
Ménétrier disease is diagnosed by endoscopy, and biopsy of stomach tissue.
Treatment may include anti-emetics, analgesics and a high-protein diet.
Part or all of the stomach may need to be removed if the disease is severe.
Cetuximab (Erbitux) blocks the action of TGF-α and is being investigated as a promising new treatment for Ménétrier disease.
Markedly hypertrophic rugae resembling cerebral convolutions often centered along the greater curvature of the stomach.
Transition between normal and diseased mucosa always abrupt.
Microscopically there is marked hyperplasia of the crypts accompanied by mild atrophy of the underlying secretory mucosa