Clinical characteristics, treatment outcomes, and relapse prediction in 199 patients with generalized pustular...

Generalized pustular psoriasis (GPP) is a rare, life-threatening inflammatory disease with recurrent flares and clinical heterogeneity. Long-term management remains challenging due to limited evidence on treatment efficacy and prognosis.

This study sought to optimize clinical management of GPP by evaluating treatment efficacy, prognostic factors including genetics, and developing a practical tool for relapse prediction.

This study involved 199 GPP patients, analyzing clinical, genetic, and treatment data. Treatment efficacy was evaluated using the Cox proportional hazards model. Multivariate logistic regression identified relapse predictors, incorporated into a nomogram and online tool. Model performance was evaluated using the area under the curve, calibration curves and decision curve analysis.

IL36RN mutations were linked to earlier onset and increased systemic involvement. Biologic therapies, especially IL-36 inhibitors, showed better results than conventional treatments, with shorter hospital stays and faster pustule clearance. Biologics reduced relapse risk by 95%. Relapse predictors included a history of plaque psoriasis, elevated direct bilirubin (DBIL > 8 µmol/L), and biologic use. The relapse prediction model had good discrimination (AUC = 0.75) and stratified patients into high-, intermediate-, and low-risk groups (high-risk vs. low-risk: 9.51-fold higher recurrence).

Biologics, particularly IL-36 inhibitors, improve GPP outcomes. DBIL is a novel biomarker for relapse risk. The validated online tool provides a practical way to personalize relapse prevention and treatment in clinical practice.