Patients with a homozygous loss-of-function mutation in OTULIN develop OTULIN-related autoinflammatory syndrome (ORAS), a potentially fatal autoinflammatory condition which can be managed by treatment with anti-TNF neutralizing antibodies [9, 10, 12]. Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.