Generalized Pustular Psoriasis: Current Treatment and Innovative Therapies

Area of Science:

• Dermatology
• Immunology
• Genetics

Background:

• Generalized pustular psoriasis (GPP) is a rare, severe auto-inflammatory condition.
• IL-36 pathway dysregulation drives GPP flares, causing erythema, pustules, and systemic illness.
• Real-world data on spesolimab, a key GPP therapy, is limited, especially outside Europe.

Purpose of the Study:

• To describe the clinical characteristics of GPP patients in Latin America.
• To evaluate the efficacy and safety of spesolimab in a real-world setting.
• To propose a treatment algorithm for GPP flares.

Main Methods:

• Retrospective case series with prospective follow-up (2010-2025).
• Nine patients diagnosed with GPP according to ERASPEN criteria were included.
• Data collected included demographics, triggers, clinical features, complications, and treatments.

Main Results:

• Nine patients (3-61 years) with GPP were analyzed; obesity and steroid withdrawal were common.
• All patients experienced flares with erythema, pustules, and pain; complications included ARDS and cholangitis.
• Spesolimab achieved 100% flare-free status at 12 months, outperforming non-IL-36 agents.

Conclusions:

• The largest Latin American GPP cohort shows earlier onset in pediatric cases and high complication rates.
• Spesolimab demonstrates superior flare control, but access remains a challenge.
• Standardized protocols and genetic research in diverse populations are crucial for GPP management.