Clinical Practice Recommendations for Non-Dermatologists on the Diagnostic Suspicion of GPP

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening, chronic cutaneous inflammatory disease characterized by unpredictable, recurrent acute flares of painful sterile pustules on a widespread erythematous background. In addition to cutaneous manifestations, patients may experience fever, pruritus, pain, chills, and general malaise, which may be further complicated by secondary infection, sepsis, and organ failure, thus requiring urgent medical treatment and, in some cases, hospitalization. Prompt therapeutic management of the acute phase is crucial for severe cases, and proactive treatment to prevent flares should always be considered. However, early recognition of acute flares can be challenging due to the low frequency of the disease, the rapid onset of flares, the lack of hematological biomarkers and the absence of standardized diagnostic criteria. Moreover, despite the approval of new targeted therapies, there are still several unmet needs, as these treatments are highly expensive, not always readily available, and may have limited efficacy in patients with advanced or complicated disease. For these reasons, multidisciplinary round-table discussions and shared diagnostic and therapeutic algorithms involving dermatologists, who are responsible for diagnosing and treating GPP, and other medical specialists are desirable to facilitate prompt referral to dermatologists for accurate diagnosis and appropriate treatment. We report the updated literature discussed during a multidisciplinary meeting with the aim of providing practice recommendations for clinicians involved in GPP management.