Additionally, genetics is an essential factor contributing to syndromes that associate PG and autoinflammatory phenomena, as has been verified with mutation in the Proline-Serine-Threonine Phosphatase Interactive Protein (PSTPIP)-1 gene that encodes for the CD2-binding protein 1, located on chromosome 15q, which is involved in PG and associated autoinflammatory syndromes such as PAPA syndrome (pyogenic sterile arthritis, PG, and cystic acne) and PASH syndrome (PG, acne, and suppurative hidradenitis) [22,23]. This evidence concerns the gene PSTPIP1 and Pyoderma gangrenosum-acne-suppurative hidradenitis syndrome.