A patient with pyoderma gangrenosum, acne and suppurative hidradenitis has an autoinflammatory syndrome referred to as the PASH syndrome.
Features of PASH (triad):
(1) pyoderma gangrenosum
(2) acne
(3) hidradenitis suppurativa
Features that should be absent:
(1) arthritis (presence favors PAPA or PASS syndromes)
(2) mutations of the PSTPIP1 gene (presence seen in PAPA syndrome)
A patient with PASH syndrome may respond to interleukin 1-beta blockade.
