Ulcerative jejunoileitis and enteropathy-associated T-cell lymphoma

Ulcerative jejunoileitis and enteropathy-associated T-cell lymphoma are rare conditions described in patients with refractory coeliac disease. Ulcerations affect the small bowel and are unrelated to drugs, ischaemia, infections or other known causes. We describe a female patient with an unclassified enteropathy who experienced several episodes of jejunoileal ulcerations. Several resections of the small bowel segments were necessary. The repetitive ulcerations were either from cytotoxic T cells, the patient developed a T-cell lymphoma, and malignant cells could be detected at the bottom of the ulcers, or from acid-producing cells in areas of gastric metaplasia. Two mechanisms might thus be responsible for the occurrence of repetitive ulceration, and require different treatment strategies. The patient is currently being treated with proton pump inhibitors, oral steroids and parenteral nutrition.