The two main syndromes including HS as a part of their cutaneous manifestations are PASH, a disorder presenting with the triad pyoderma, acne and HS (72–76), and PAPASH, a syndrome described by our group and characterized by the same triad of PASH and pyogenic arthritis (77) in whom genetic studies evaluating exons 10 and 11 of the PSTPIP1 gene revealed a p.E277D previously unreported missense mutation. Here, PSTPIP1 is linked to histiocytic sarcoma.