Gastrointestinal involvement in systemic mastocytosis

Abstract

Out of 4 consecutive patients with systemic mastocytosis, one had a malabsorption syndrome with only minor histologic changes of the intestinal mucosa. Another patient with ulcer diathesis had a gastric secretory pattern resembling Zollinger Ellison syndrome. Serum gastrin and histamine levels were consistently normal in all patients. Endoscopy of stomach and colon disclosed urticaria like papulae either spontaneously or after topical provocation in all patients. No increase of mast cells was found in multiple mucosal biopsies. A markedly increased gastric tissue content of histamine was found, however, in the 3 patients studied. The findings suggest that urticaria like lesions associated wih a high tissue content of histamine may be more important that hyperhistaminemia in causing the various gastrointestinal symptoms.