Interestingly, homozygous loss-of-function mutations in the OTULIN gene cause an auto-inflammatory condition, called ORAS (OTULIN-related inflammatory syndrome) or otulipenia that is responsive to anti-TNF treatment (Damgaard et al., 2016; Zhou et al., 2016). Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.