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Superficial Neutrophilic Dermatoses: From Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Disease) to Intercellular IgA Dermatoses

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Neutrophilic Dermatoses

Abstract

Since the initial description of the subcorneal pustular dermatosis by Sneddon and Wilkinson in 1956 [1], our understanding of the pathophysiology of diseases associated with the accumulation of neutrophils in the epidermis and intraepidermal blistering has significantly evolved. Sneddon-Wilkinson disease is now regarded as condition belonging to the spectrum of neutrophilic dermatoses characterized by neutrophilic infiltration of the skin and potential extracutaneous involvement, such as Sweet syndrome and pyoderma gangrenosum [2–4]. This group of inflammatory conditions, which show considerable clinical and histological overlap, is frequently associated with systemic diseases. Recently, it has been suggested to use the term of superficial neutrophilic dermatoses for all neutrophilic diseases associated with intraepidermal neutrophil accumulation [5]. Intriguingly, there is a group of patients who develop a superficial neutrophilic disease very similar to Sneddon-Wilkinson disease, showing either subcorneal or intraepidermal pustule formation. However, in contrast to Sneddon-Wilkinson disease, these patients show characteristically IgA deposits on the cell surface of epidermal keratinocyte in a pemphigus-like pattern or more rarely linear deposits in the subcorneal zone.

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Acknowledgments

We thank Dr. Michael Horn, Head Autoimmunediagnostics, University Institute of clinical chemistry, Inselspital, Bern University Hospital, University of Bern, Switzerland, for the immunofluorescence pictures.

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Authors and Affiliations

  1. Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland

    Laurence Feldmeyer & Luca Borradori

  2. Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan

    Takashi Hashimoto

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  1. Laurence Feldmeyer
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  2. Takashi Hashimoto
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Correspondence to Laurence Feldmeyer .

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  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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Feldmeyer, L., Hashimoto, T., Borradori, L. (2018). Superficial Neutrophilic Dermatoses: From Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Disease) to Intercellular IgA Dermatoses. In: Wallach, D., Vignon-Pennamen, MD., Valerio Marzano, A. (eds) Neutrophilic Dermatoses. Springer, Cham. https://doi.org/10.1007/978-3-319-72649-6_9

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