Journal
BIOMEDICINES
Volume 9, Issue 12, Pages -Publisher
MDPI
DOI: 10.3390/biomedicines9121746
Keywords
pustular psoriasis; generalized pustular psoriasis; palmoplantar pustular psoriasis; palmoplantar pustulosis; impetigo herpetiformis; acrodermatitis continua of Hallopeau; clinical features; pathogenesis; therapy
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Pustular psoriasis is a clinicopathological entity with different variants characterized by sterile pustules on an erythematous base, showing intense inflammatory response and involvement of the IL-36 axis. Due to the rarity and heterogeneity of PP forms, data on prognosis and therapeutic management are limited, leading to ongoing debates on the diagnosis and treatment of PP.
Pustular psoriasis (PP) is a clinicopathological entity encompassing different variants, i.e., acute generalized PP (GPP), PP of pregnancy (impetigo herpetiformis), annular (and circinate) PP, infantile/juvenile PP, palmoplantar PP/palmoplantar pustulosis, and acrodermatitis continua of Hallopeau (ACH), which have in common an eruption of superficial sterile pustules on an erythematous base. Unlike psoriasis vulgaris, in which a key role is played by the adaptive immune system and interleukin (IL)-17/IL-23 axis, PP seems to be characterized by an intense inflammatory response resulting from innate immunity hyperactivation, with prominent involvement of the IL-36 axis. Some nosological aspects of PP are still controversial and debated. Moreover, owing to the rarity and heterogeneity of PP forms, data on prognosis and therapeutic management are limited. Recent progresses in the identification of genetic mutations and immunological mechanisms have promoted a better understanding of PP pathogenesis and might have important consequences on diagnostic refinement and treatment. In this narrative review, current findings in the pathogenesis, classification, clinical features, and therapeutic management of PP are briefly discussed.
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