Generalized pustular psoriasis (GPP) is a severe and life-threatening systemic disease associated with significant morbidity and mortality. Recent progress has been made in understanding the pathogenetic pathways involved in GPP and an intricate interaction between innate and adaptive immune mechanisms has been suggested. Despite formal consensus guidelines on pustular psoriasis currently available in the literature, the definitions and classifications of GPP used across studies were inconsistent. Consequently, there are no unified criteria that can be universally adopted for precise diagnosis, classification and effective treatment of GPP patients with new targeted drugs. The aim of this review was to collect all the main evidence on available diagnostic criteria for GPP and to establish recommendations in order to promote a better stratification and therapeutic management of this severe and heterogeneous disease.