Abstract
PAPASH syndrome, a rare autoinflammatory condition characterized by pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa, presents significant treatment challenges due to its rarity and complex multisystem involvement. Since its initial description in 2013, only 14 cases have been documented, leading to limited treatment experience. Although IL-1 and TNF-alpha blocking agents have shown efficacy, responses vary due to genetic and pathogenetic differences, with some cases being resistant. Therefore, alongside summarizing prior treatment experiences, new treatment modalities need to be explored. This report presents the case of a 46-year-old Native American male with PAPASH syndrome who responded successfully to IL-17 inhibition with secukinumab. The patient experienced marked improvement in both dermatologic and rheumatologic symptoms, highlighting the potential role of IL-17 in the pathogenesis of PAPASH. This case suggests that IL-17 inhibition could be a promising treatment modality for PAPASH syndrome.