In line with the role of OTULIN in counteracting LUBAC, OTULIN-deficient skin fibroblasts from patients with ORAS showed reduced TNF-induced NF-κB and MAPK activation but enhanced sensitization to TNF-induced cell death relative to healthy controls (29). Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.