Abstract
Background: Generalized pustular psoriasis (GPP) is a diagnostic challenge for clinicians and is relatively rare. Prevalence is currently reported to be higher in Asian countries and among female patients; peak age of onset is variable across studies. Objective: This article provides clinicians with an overview of the global prevalence of GPP, common clinical presentation and diagnostic criteria, pathogenesis, and evolving treatment options. Discussion: Clinical presentation is characterized by onset of generalized sterile pustules with erythema, and may include systemic inflammatory signs including fever and lab abnormalities. Diagnostic criteria vary globally. European Rare and Severe Psoriasis Expert Network (ERASPEN) criteria present a clinical diagnosis while Japanese criteria include a biopsy. Advancements in understanding of pathogenesis have uncovered the role of IL-36 as a driver of disease, and this is the target of new treatments including spesolimab, an anti-IL-36 receptor antibody.
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