Investigation of 7 cases of PASH and 13 cases of isolated PG revealed multiple mutations in a variety of autoinflammatory genes, including PSTPIP1, Mediterranean fever (MEFV), Nucleotide-binding oligomerization domain-containing protein 2 (NOD2), NLRP3, NLRP12, Lipin 2 (LPIN2) (2). Here, NOD2 is linked to Pyoderma gangrenosum-acne-suppurative hidradenitis syndrome.