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Clinical Reviews in Allergy & Immunology

, Volume 53, Issue 3, pp 428–438 | Cite as

Cutaneous Manifestations of ANCA-Associated Small Vessels Vasculitis

  • Angelo Valerio Marzano
  • Maria Gabriella Raimondo
  • Emilio Berti
  • Pier Luigi MeroniEmail author
  • Francesca Ingegnoli
Article

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.

Keywords

Anti-neutrophil cytoplasmic antibodies Vasculitis Skin Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis Microscopic polyangiitis 

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

Notes

Compliance with Ethical Standards

Conflicts of Interest

The authors declare that they have no conflict of interest.

Funding

None

Ethical Approval and Informed Consent

Not necessary

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© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Unità Operativa di Dermatologia, IRCCS Ca’ Granda, Ospedale Maggiore PoliclinicoUniversità degli Studi di MilanoMilanItaly
  2. 2.Division of Rheumatology, ASST Pini, Department of Clinical Sciences and Community HealthUniversità degli Studi di MilanoMilanItaly
  3. 3.Experimental Laboratory of Immunological and Rheumatologic ResearchesIRCCS Istituto Auxologico ItalianoMilanItaly

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