Abstract
Introduction: Generalized pustular psoriasis (GPP) is a rare, chronic inflammatory skin disease characterized by persistent symptoms and sudden flares of painful sterile pustules, sometimes accompanied by systemic inflammation. Patients with GPP experience chronic disease burden even when not experiencing flares. There is an unmet need for guidelines on continuous long-term management of this disease. Areas Covered: This review summarizes existing literature describing the chronic disease burden of GPP, the persistence of symptoms and effects on quality of life (QoL) when patients are not experiencing a flare, the recurring nature of GPP flares, and the high prevalence of chronic comorbidities. We also present an overview of results from the EFFISAYIL® 2 study, which was the first randomized, placebo-controlled clinical trial to systematically evaluate continuous management with subcutaneous spesolimab, a first-in-class anti-interleukin-36 receptor monoclonal antibody specifically designed to treat GPP. Expert Opinion: An unmet need in GPP is the establishment of guidelines for chronic disease management, including measures for treating GPP between flares, flare prevention, and long-term disease control. Treatment strategies should mitigate both the persistent disease burden and potentially life-threatening flare episodes. Intravenous spesolimab is currently the only FDA-approved medication to treat GPP flares, and subcutaneous spesolimab is the only FDA-approved medication to treat GPP when patients are not experiencing a flare. Guidelines should aim to advance the recognition of GPP as a chronic disease and emphasize prompt diagnosis and timely access to FDA-approved therapies according to the diagnostic criteria established by the International Psoriasis Council and the National Psoriasis Foundation.