Free perforation due to intestinal lymphoma in biopsy-defined or suspected celiac disease

Malignant complications occur in celiac disease and dermatitis herpetiformis, particularly small bowel lymphoma. Although intestinal ulceration, especially in jejunum or ileum is rare, spontaneous free perforation of the intestine developed in 6 patients with established or suspected celiac disease and dermatitis herpetiformis due to a malignant lymphoma. In 3 cases, perforation developed after celiac disease was diagnosed and compliance with a gluten-free diet was strictly maintained and repeatedly evaluated. In 1 of these cases, refractory celiac disease was defined. In another, free perforation was the presenting manifestation of the lymphoma and this led to a later diagnosis of subsequent biopsy-defined latent celiac disease. In 2 others, celiac disease was suspected but the rapidly deteriorating clinical course prohibited institution of a gluten-free diet. In all patients, the lymphoma involved the jejunum, but in 1 of these, perforation was defined in the colon indicating that any intestinal site may suffer free perforation from lymphoma in celiac disease. This report documents a high mortality for this serious complication in celiac disease, its lack of predictability and the potential for development even with strict compliance to a gluten-free diet.