In humans, homozygous mutations in OTULIN cause OTULIN-related autoinflammatory syndrome (ORAS) (also known as otulipenia or autoinflammation, panniculitis, and dermatosis syndrome; OMIM #617099), a life-threatening autoinflammatory disease characterised by fevers, panniculitis, diarrhoea, and arthritis [31, 32, 36, 37]. Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.