PAPA syndrome (PAPAs) is an autosomal-dominant condition caused by mutations in the PSTPIP1 gene, situated at the locus 15q24-q25.1, which codifies the CD2-binding protein 1 (CD2BP1), enclosed in the pyrin-mediated inflammatory pathway [113]. Here, PSTPIP1 is linked to pyogenic arthritis-pyoderma gangrenosum-acne syndrome.