Abstract
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.
Highlights
Pustular psoriasis (PP) encompasses a heterogeneous group of nosological entities sharing cardinal clinicopathological features [1]
Network (ERASPEN), PP was classified into three distinct types: (1) generalized PP (GPP), either relapsing or persistent, that can occur with or without psoriasis vulgaris (PV) and with or without systemic inflammation; (2) palmoplantar pustulosis (PPP); (3) acrodermatitis continua of Hallopeau (ACH) [5]
Case reports documenting ACH transitioning to GPP together with the possible association with IL36RN, CARD14 and AP1S3 variants support the existence of shared disease spectrum, with ACH
Summary
Pustular psoriasis (PP) encompasses a heterogeneous group of nosological entities sharing cardinal clinicopathological features [1]. Such diseases are typified by an eruption of superficial sterile pustules, usually on an erythematous base. Histopathological features include hyperkeratosis, parakeratosis, acanthosis, diffuse dermal mononuclear and neutrophilic inflammatory infiltrates, intraepidermal collections of neutrophils, rete ridge elongation and dilated tortuous vessels in the papillary dermis [2,3]. The relative rarity and heterogeneity of PP forms have hampered the collection of precise data on prognosis and management. Recent progresses in the identification of genetic mutations and immunological mechanisms have led to a better understanding of PP pathogenesis and might have relevant consequences on diagnostic refinement and treatment
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