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.


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