A rare case of Crohn's ileitis in a patient with constitutional mismatch repair deficiency
Constitutional mismatch repair deficiency (CMMRD), a variant of Lynch syndrome, is a rare disease characterized by cafÃ©-au-lait spots, oligopolyposis, glioblastoma and lymphoma. A 24-yearold male, under surveillance for CMMRD, developed Crohn's ileitis after total colectomy with end ileostomy for colorectal cancer and failed to respond to oral corticosteroids. The patient underwent induction and maintenance of remission with vedolizumab infusions. We report the first patient with CMMRD developing Crohn's disease. The choice of immunosuppressive therapy in these patients is challenging and needs to be made according to their risk for malignancy.
Keywords Crohn's disease, familial cancer, vedolizumab, constitutional mismatch repair deficiency
Ann Gastroenterol 2017; 30 (3): 370-372