NOD2 and familial Mediterranean fever: Indeed, in addition to the involvement of the “paradigmatic” gene PSTPIP1 (serine-threonine phosphatase interacting protein 1), pathogenic variants in autoinflammatory genes such as MEFV (mediterranean familial fever), NLRP (NOD-like receptor family pyrin domain containing) 3/12, NOD2 (nucleotide binding oligomerization domain containing 2 genes) and IL1RN (interleukin 1 receptor antagonist), leading to an exacerbated release of IL-1β, have been reported in both syndromic (PAPA, PASH and PAPASH) and sporadic PG [18].