Since OTULIN is known to regulate NF-κB signaling and cell death responses [7, 11], and since clinical manifestations in ORAS patients include intestinal abnormalities [9, 10, 12, 28], we here investigated the role of OTULIN in the intestinal epithelium. Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.