IPEX is a life-threatening condition, with some patients developing AID already in fetal life and rarely surviving infancy in the absence of hematopoietic stem cell transplantation (HSCT); type I diabetes, autoimmune enteropathy leading to chronic diarrhea, inflammatory bowel disease (IBD), and cytopenias are the most common autoimmune manifestations; affected infants also present severe allergy and high IgE levels (4, 5). Here, IGHE is linked to inflammatory bowel disease.