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Pustular Psoriasis

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Neutrophilic Dermatoses

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Abstract

Pustular forms of psoriasis are a group of rare, debilitating, and often life-threatening inflammatory skin diseases characterized by episodic skin infiltration of neutrophils, pustule development, erythema and desquamation, which can manifest in the presence or absence of chronic plaque psoriasis. The skin involvement is frequently accompanied by a high-grade fever, fatigue and systemic inflammation. Despite having different genetic and environmental etiologies generalized pustular psoriasis (GPP), acute generalized exanthemous pustulosis (AGEP), palmo-plantar pustulosis (PPP) and acrodermatitis continua of Hallopeau (ACH) are all characterized by a massive influx of neutrophils into the epidermis, thus share some mechanistic aspects. To date, treatments for these diseases have been unsatisfactory with disease recurrence common; however, recent genetic data and ongoing clinical trials with biologic therapies are revealing hitherto unknown mechanisms, substantially improving our understanding and management of these diseases.

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Authors and Affiliations

  1. Department of Dermatology, University of Michigan, Ann Arbor, MI, USA

    Andrew Johnston

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  1. Andrew Johnston
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Correspondence to Andrew Johnston .

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Editors and Affiliations

  1. Physician (Hon.) of Paris Hospitals, Paris, France

    Daniel Wallach

  2. Pathology Department, APHP Hôpital Saint Louis, Paris, France

    Marie-Dominique Vignon-Pennamen

  3. Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Universita’ degli Studi di Milano, Unita’ Operativa di Dermatologia, IRCCS Fondazione Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy

    Angelo Valerio Marzano

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Johnston, A. (2018). Pustular Psoriasis. In: Wallach, D., Vignon-Pennamen, MD., Valerio Marzano, A. (eds) Neutrophilic Dermatoses. Springer, Cham. https://doi.org/10.1007/978-3-319-72649-6_11

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  • DOI: https://doi.org/10.1007/978-3-319-72649-6_11

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