Exploring the Clinical Assessment, Guidelines, and Options for the Treatment of Generalized Pustular Psoriasis...

Acute episodes of generalized pustular psoriasis (GPP), known as "flares", are characterized by the widespread appearance of pustules with surrounding skin erythema, and are often accompanied by systemic symptoms. The clinical course of GPP is unpredictable, and symptoms vary in extent and severity; the disease may be relapsing-remitting with recurrent episodes of pustulosis, or be more persistent. The triggers that may lead to flares include withdrawal of corticosteroids, stress, pregnancy, and infections. GPP-specific assessment tools, such as the Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) and the General Pustular Psoriasis Area and Severity Index (GPPASI), were developed to evaluate the severity of disease, and to monitor the patient's response to therapy during clinical trials. Spesolimab is the first GPP-specific treatment available in the United States for the treatment of GPP flares in adults, and was approved by the US FDA in September 2022. To date, spesolimab has been approved by regulatory agencies in almost 40 countries, including Japan, Mainland China, and the European Union. Spesolimab is a first-in-class humanized monoclonal antibody that targets the interleukin-36 receptor, and blocks the downstream effects of the interleukin-36 pathway, which is associated with GPP pathogenesis. Data from clinical trials demonstrate the safety and efficacy of spesolimab in providing rapid clinical improvement for patients with GPP flares. Standardized international guidelines for the diagnosis and management of GPP are needed, and no recent GPP guidelines are available in the US. This podcast discusses clinical assessment tools for GPP (GPPGA and GPPASI), the evolution of GPP management guidelines, the therapeutic landscape of GPP, efficacy and safety data for spesolimab, and examines important considerations for patients living with this condition.