Literature review and expert opinion on diagnosis and current management of generalized pustular psoriasis

Generalized pustular psoriasis (GPP) is a rare, chronic, systemic, autoinflammatory disease characterized by the eruption of sterile pustules, often accompanied by more general symptoms, such as fever, fatigue, and a burning sensation in the skin. GPP can be potentially life-threatening, if untreated, as it can lead to complications, such as sepsis and heart failure.

In this literature review and expert opinion article, we provide an overview of the epidemiology, pathogenesis, clinical presentation, diagnosis, and treatment of GPP. Eleven dermatologists representing seven different Italian regions considered relevant evidence in the literature to discuss the current diagnosis and treatment of GPP. The expert panel of dermatologists identified several weaknesses in the current clinical management of GPP.

There is an inconsistent definition and classification of the disease across the literature, which can lead to misdiagnosis and delay in disease treatment. Furthermore, there are no international and standardized clinical guidelines on disease management, especially in Europe. There is a profound need for the development of novel therapeutic agents with sustained efficacy to decrease the impact of the comorbidities and mortality associated with GPP, prevent the onset of complications, and support the unmet needs of these patients.