Management of generalized pustular psoriasis

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening autoinflammatory skin disease characterized by recurrent flares of sterile pustules on widespread erythema.Acute flares often accompany severe systemic symptoms like high fever and fatigue, carrying a significant mortality risk due to complications such as septic shock or cardiorespiratory failure.Recent advances in molecular pathophysiology have established the central role of IL-36 signaling pathway in GPP pathogenesis, involving an aberrant IL-1/IL-36-chemokine-neutrophil pathogenic axis, frequently associated with genetic predispositions, notably loss-of-function mutations in IL36RN.International consensus criteria, recently established by the International Psoriasis Council, emphasizes the critical need for timely clinical diagnosis based on macroscopic findings and careful differentiation from clinical mimics like acute generalized exanthematous pustulosis.Based on the current pathophysiologic understanding, novel targeted therapies, particularly IL-36 receptor antagonists, have demonstrated rapid and marked efficacy in controlling acute GPP flares, marking a new therapeutic era.Given the chronic and highly relapsing nature of GPP, successful management requires both aggressive acute flare intervention to immediately resolve systemic inflammation and avert life-threatening complications, and comprehensive, sustained longterm relapse prevention.Continuous research is required to develop individualized treatment strategies that reflect GPP' s clinical heterogeneity and optimize patient outcomes.