Abstract
Generalized pustular psoriasis (GPP) is a rare and severe auto‑inflammatory skin disease that is characterized by recurrent, acute onset, and generalized pustular eruptions on erythematous, inflamed skin. GPP is traditionally classified as a variant of psoriasis vulgaris, even though recent clinical, histological and genetic evidence suggests that it is a heterogeneous disease and requires a separate diagnosis. In recent years, variants of IL36RN, CARD14, AP1S3 and MPO genes have been identified as causative or contributing to genetic defects in a proportion of patients affected by GPP. These disease‑related genes are involved in common inflammatory pathways, in particular in the IL‑1/IL‑36‑chemokines‑neutrophil pathogenic axis. At present, no standard therapeutic guidelines have been established for GPP management, and there is a profound need for novel efficacious treatments of GPP. Among them, biological agents antagonizing the IL‑36 pathway are promising therapeutics. The aim of the present review is to provide the most recent updates on the genetics, genotype‑phenotype correlation and pathological basis of GPP, as well as on biologic treatments available for GPP and relative clinical courses.