Evidence on Hidradenitis Suppurativa as an Autoinflammatory Skin Disease

Hidradenitis Suppurativa (HS) is characterized as an autoinflammatory skin disease involving genetic predispositions, immune dysregulation, and environmental factors. The review emphasizes the role of innate immunity dysregulation and autoinflammation, particularly the IL-1 pathway and NLRP3 inflammasome, in HS pathogenesis. Genetic mutations in genes like NCSTN, PSENEN, and PSEN1 contribute to the disease, with elevated inflammatory cytokines such as IL-1β, IL-36, and IL-8 observed in lesions. Despite treatments like adalimumab benefiting only 40% of patients, investigational therapies targeting IL-1 pathways show promise. HS is linked to autoinflammatory syndromes like PASH, highlighting the need for further research to develop targeted therapies.