Generalized pustular psoriasis is a rare, severe, and potentially life-threatening inflammatory skin disease characterized by the acute or recurrent development of sterile pustules on a background of generalized erythema, frequently accompanied by systemic manifestations.

Its distinct pathogenesis, driven primarily by excessive activation of the interleukin-36 pathway and the innate immune response, differentiates generalized pustular psoriasis from plaque psoriasis and necessitates a specific diagnostic and therapeutic approach. The rarity of the disease, the limited number of randomized clinical trials, and differenced among available recommendations have contributed to considerable variability in clinical practice. Recent advances in understanding of its pathophysiology and the development of targeted therapies, particularly interleukin-36 receptor inhibitors, have substantially transformed the management of this condition.

This paper presents diagnostic and therapeutic recommendations for generalized pustular psoriasis developed by experts of the Polish Dermatological Society on the basis of available scientific evidence, international consensus documents, and clinical experience. The recommendations address the current definition and diagnostic criteria, epidemiology, pathogenesis, histopathological features, and principles of disease severity assessment using validated evaluation tools. Particular attention is given to differential diagnosis, the role of systemic manifestations, and associated comorbidities. Therapeutic goals during acute flares and long-term management are discussed together with currently available treatment options, with particular emphasis on therapies targeting the interleukin-36 pathway.

The aim of these recommendations is to standardize clinical management, improve patient safety, and optimize treatment outcomes in this severe form of psoriasis.