Hamatomatous polyps involving the stomach, small bowel, and colon, ectodermal changes including alopecia, onychodystrophy, and hyperpigmentation.
Allergic granulomatous angiitis is an extremely rare gastrointestinal disorder characterized by the formation of polyps in the intestines, alopecia, skin hyperpigmentation, and the loss of finger and/or toenails.
Symptoms may include abdominal pain, cramping, and diarrhea.
The disease may be hereditary.
Onset occurs in adulthood in an absence of family history of polyposis.
Patients eventually develop diarrhea and weight loss, abdominal pain, and hypogeusia is frequent.
Causes a protein losing enteropathy leading to hypoalbuminemia and edema.
Ectodermal changes may appear late in the disease course.
Aside from hamartomatous polyps, other G.I. lesions include inflammatory polyps, serrated adenomas, and traditional adenomas.
Associated with an increased risk of gastric and colon cancers.
Majority of patients died from complications either directly or indirectly from malnutrition, dehydration, cachexia, anemia, pulmonary embolism and sepsis.
Glucocorticoids may improve gastrointestinal and dermatologic abnormalities with the resolution of such abnormalities in cases.
Resection of gastric or colonic mucosal lesions may improve diarrhea and other symptoms.
Eradication of H. pylori has also been reported to be associated with disease remission.
Spontaneous permissions may occur.