In the same year, another group identified three more patients, from three different consanguineous families, carrying OTULIN biallelic mutations and symptoms of systemic sterile inflammation (e.g., prolonged fevers and diarrhoea) that they called Otulipenia [63] (Table 1). Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.