Introduction:

PASH (pyoderma gangrenosum, acne, suppurative hidradenitis) syndrome is a rare autoinflammatory disorder that combines pyoderma gangrenosum, acne and hidradenitis suppurativa, linked to increased CCTG repeats in the PSTPIP1 gene promoter.

Objective:

To describe successful treatment of PASH syndrome with spesolimab and review of the literature on the treatment of PASH syndrome.

Case report:

A 38-year-old woman with a history of severe acne and chronic hidradenitis suppurativa presented with a rapidly worsening ulcer on her left lower leg, diagnosed as pyoderma gangrenosum. This triad of symptoms confirmed the diagnosis of PASH syndrome. Initial treatment with dexamethasone, cyclosporine, clindamycin and rifampicin yielded only temporary symptoms relief. The patient participated in a clinical trial involving spesolimab which resulted in complete remission of pyoderma gangrenosum and marked improvement in hidradenitis suppurativa and acne; remission was maintained for 1.5 years.

Conclusions:

PASH syndrome exhibits recurrent flares and a complex clinical course, often with reduced treatment efficacy over time. Spesolimab, by targeting IL-36-mediated inflammation, shows promise as a focused and highly effective treatment.