IL10-related early-onset inflammatory bowel disease
IL10-related early-onset inflammatory bowel disease
Definition
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.
Also known as IL10-related early-onset IBD, IL10-related early-onset inflammatory bowel disease, immune dysregulation-inflammatory bowel disease-arthritis-recurrent infections syndrome — per MONDO