GPP: What APPs Should Know
This narrative review summarizes the current understanding of generalized pustular psoriasis (GPP), a rare but potentially life-threatening inflammatory skin disease marked by widespread sterile pustules and often systemic inflammation. The authors emphasize that GPP can overlap with plaque psoriasis but is increasingly recognized as a distinct condition with different clinical, genetic, and immune features. The IL-36 pathway plays a central role in GPP pathogenesis, and mutations such as those involving IL-36RN may contribute to disease development. Diagnosis requires careful evaluation of clinical presentation, triggers, patient history, laboratory findings, and, when appropriate, histopathology.
The review also highlights the seriousness of GPP flares, which may involve fever, malaise, elevated inflammatory markers, electrolyte abnormalities, kidney or liver dysfunction, sepsis, or multi-organ failure. Management historically has relied on treatments used for plaque psoriasis, but newer targeted approaches have emerged as understanding of IL-36 signaling has improved. Spesolimab is identified as the only therapy specifically approved for GPP flares in adolescents and adults in both Europe and the United States. The authors conclude that GPP remains complex to diagnose and manage, and that standardized international guidance and continued research are needed to improve care.
Reference: Torres T, Antunes J, Tavares Bello R, et al. Update on Generalized Pustular Psoriasis. Acta Med Port. 2025 May 2;38(5):321-330. doi: 10.20344/amp.22672. Epub 2025 May 2. PMID: 40359115.
Tristan Hasbargen
PA-C