TNFAIP3 and autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive: Recently, deregulations in the UPS were reported in patients with autoinflammatory disorders including chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (13), linear ubiquitin chain assembly complex (LUBAC) deficiency (14, 15), haploinsufficiency of A20 (HA20) (16), and otulipenia/otulin-related autoinflammatory syndrome (ORAS) (17, 18).