Generalized pustular psoriasis: practical recommendations for Spanish primary care and emergency physicians

Abstract

Generalized pustular psoriasis (GPP) is a rare chronic inflammatory skin disease that can lead to life-threatening complications and require emergency medical treatment. Recurrent GPP flares are characterized by the sudden onset of widespread erythematous skin rash with sterile pustules, at times associated with fever, chills, general malaise, and other systemic inflammatory manifestations. Systemic complications such as cardiorespiratory failure, infections, and sepsis are potentially life-threatening and can result in an emergency department visit and/or hospitalization. Acute GPP episodes can be difficult to recognize and diagnose. The low incidence of the disease, its relapsing nature, the unpredictability of flare onset, and the lack of standardized diagnostic criteria are major obstacles to achieving rapid recognition and diagnosis in both the emergency department and the hospital setting. There is scarce evidence supporting the efficacy and safety of treatments commonly used for GPP; consequently, there is an unmet need for therapies that specifically target the condition. Our aim is to present a multidisciplinary approach to GPP to achieve a rapid diagnosis ensuring that the patient receives the most appropriate treatment for their pathology. The main recommendation for primary care and emergency physicians is to contact a dermatologist immediately for advice or to refer the patient when GPP or a flare is suspected.