A rare immune dysregulation disease with immunodeficiency characterized by severe, progressive infantile onset inflammatory bowel disease with pancolitis, perianal disease (ulceration, fistulae), recurrent respiratory, genitourinary and cutaneous infections, arthritis and a high risk of B-cell lymphoma. The disease is MONDO_0016542 (IL10-related early-onset inflammatory bowel disease). Also known as: IL10-related early-onset IBD, immune dysregulation-inflammatory bowel disease-arthritis-recurrent infections syndrome.