Pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome with recurrent vasculitis

Abstract

Autoinflammatory syndromes are characterized by recurrent episodes of sterile inflammation in the absence of circulating autoantibodies or autoreactive T cells. PASH syndrome is a recently identified hereditary autoinflammatory syndrome consisting of multiple neutrophilic dermatoses including pyoderma gangrenosum (PG), acne, and hidradenitis suppurativa (HS). The triad of PG, acne, and HS occurs in extremely rare instances. To our knowledge, the clinical tetrad of PG, acne, HS, and vasculitis has not been reported.