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Cutaneous Manifestations of ANCA-Associated Small Vessels Vasculitis

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Abstract

Skin lesions are frequent manifestations of underlying systemic conditions, including systemic autoimmune vasculitis. In particular, anti-neutrophil cytoplasmic antibodies (ANCA) are associated with distinct forms of vasculitis characterized by inflammatory cell infiltration of the walls of small and medium-sized vessels leading to vascular destruction and tissue necrosis. ANCA-associated vasculitis is rare and systemic diseases, which can be classified based on different distribution of vascular inflammation and presence or absence of granulomatosis and asthma. Despite their diversities, ANCA-associated vasculitis, namely microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis, can all display a broad variety of cutaneous manifestations, which can appear during the course of the disease or even as first sign at the time of onset. Different skin manifestations might coexist in the same patient and occur in different occasions during the course of the vasculitis. Thus, a deep knowledge of the spectrum of skin lesions as part of ANCA-associated vasculitis is mandatory for a correct diagnostic process, whenever cutaneous vasculitis is suspected. Due to this broad variety of manifestations, the diagnosis of skin involvement in ANCA-associated vasculitis is very challenging and it must be supported by a detailed medical history, accurate physical examination, specific histopathological analysis of skin biopsy and the presence of ANCA serology. In this review, we focus on the cutaneous manifestations that can develop in the context of ANCA-associated vasculitis, detailing the clinical features, the histopathological aspects as well as the direct immunofluorescence studies for each of the three conditions. Moreover, we acknowledged the differential diagnoses that must be ruled out in the diagnostic process and the main therapeutic approaches available for treatment of ANCA-associated vasculitis.

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Abbreviations

ANCA:

Anti-neutrophil cytoplasmic antibodies

AAV:

ANCA-associated vasculitis

PR3:

Leukocyte proteinase 3

GPA:

Granulomatosis with polyangiitis

MPO:

Myeloperoxidase

MPA:

Microscopic polyangiitis

EGPA:

Eosinophilic granulomatosis with polyangiitis

ENT:

Ear, nose and throat

CSVV:

Cutaneous small vessel vasculitis

PG:

Pyoderma gangrenosum

PAN:

Polyarteritis nodosa

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Author notes

  1. Prof. Marzano and Dr. Raimondo equally contributed to this article.

Authors and Affiliations

  1. Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Unità Operativa di Dermatologia, IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Università degli Studi di Milano, Milan, Italy

    Angelo Valerio Marzano & Emilio Berti

  2. Division of Rheumatology, ASST Pini, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy

    Maria Gabriella Raimondo, Pier Luigi Meroni & Francesca Ingegnoli

  3. Experimental Laboratory of Immunological and Rheumatologic Researches, IRCCS Istituto Auxologico Italiano, Cusano Milanino, Milan, Italy

    Pier Luigi Meroni

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  3. Emilio Berti
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  4. Pier Luigi Meroni
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  5. Francesca Ingegnoli
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Correspondence to Pier Luigi Meroni.

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Marzano, A.V., Raimondo, M.G., Berti, E. et al. Cutaneous Manifestations of ANCA-Associated Small Vessels Vasculitis. Clinic Rev Allerg Immunol 53, 428–438 (2017). https://doi.org/10.1007/s12016-017-8616-5

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