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The Extra-cutaneous Localizations of the Neutrophilic Disease

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Neutrophilic Dermatoses

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Abstract

In 1991, Vignon-Pennamen and Wallach proposed a unifying and prescient concept of “neutrophilic disease” (ND) in the light of clinical observations: (1) numerous reports of clinicopathological symptoms overlapping the typical forms of ND, including subcorneal pustular dermatosis (SPD; Sneddon-Wilkinson disease); Sweet’s syndrome (SwS); pyoderma gangrenosum (PG) and erythema elevatum diutinum (EED); (2) extracutaneous manifestations of neutrophilic disease; (3) non fortuitous association with systemic diseases and (4) usual therapeutic response to corticosteroid and/or dapsone [1]. In their article, they reviewed and argued the rare cases of various organ involvement suspected to be related to ND and previously reported, generally one or two observations for each organ, including bones, liver, lymph nodes, spleen, lung and kidney. Almost a quarter century later, a large number of extracutaneous manifestations of ND have been reported and allow a clarification on the risk of organ involvement depending on type of neutrophilic diseases, mostly Sweet syndrome and pyoderma gangrenosum, and their treatment (Table 16.1).

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Abbreviations

CRMO:

Chronic recurrent multifocal osteomyelitis

CT:

Computer tomography

DIRA:

Deficiency of interleukin-1 receptor

EED:

Erythema elevatum diutinum

GPP:

Generalized pustular psoriasis

ND:

Neutrophilic disease

NIO:

Non-infectious osteomyelitis

PG:

Pyoderma gangrenosum

SAPHO:

Synovitis, acne, pustulosis, hyperostosis and osteitis

SPD:

Subcorneal pustular dermatosis

SwS:

Sweet syndrome

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Authors and Affiliations

  1. Department of Dermatology, St Eloi Hospital, University of Montpellier, Cedex 5, France

    Didier Bessis

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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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Bessis, D. (2018). The Extra-cutaneous Localizations of the Neutrophilic Disease. In: Wallach, D., Vignon-Pennamen, MD., Valerio Marzano, A. (eds) Neutrophilic Dermatoses. Springer, Cham. https://doi.org/10.1007/978-3-319-72649-6_16

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