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

HS: hidradenitis suppurativa IL: interleukin LCV: leukocytoclastic vasculitis PAPA: pyogenic arthritis, pyoderma gangrenosum, and acne PAPASH: pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa PASH: pyoderma gangrenosum, acne, and hidradenitis suppurativa PG: pyoderma gangrenosum PsAPASH: psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis INTRODUCTION 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.