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Abdel-Halim et al. Vessel Plus 2022;6:8 Vessel Plus
DOI: 10.20517/2574-1209.2021.40

Review Open Access

Cutaneous manifestations of anti-neutrophil

cytoplasmic antibody associated vasculitis

2
1
Mona Abdel-Halim , Ayman Mahmoud , Gaafar Ragab 2
1
Department of Dermatology, Dermatopathology Unit, Faculty of Medicine, Cairo University, Cairo 11562, Egypt.
2
Department of Internal Medicine, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Cairo
11562, Egypt.
Correspondence to: Prof. Gaafar Ragab, Department of Internal Medicine, Faculty of Medicine, Cairo University, Twin Tower
D3E, 26 July corridor, Zayed City, Giza 12588, Egypt. E-mail: gragab@kasralainy.edu.eg
How to cite this article: Abdel-Halim M, Mahmoud A, Ragab G. Cutaneous manifestations of anti-neutrophil cytoplasmic
antibody associated vasculitis. Vessel Plus 2022;6:8. https://dx.doi.org/10.20517/2574-1209.2021.40
Received: 26 Feb 2021 First Decision: 6 Apr 2021 Revised: 18 Apr 2021 Accepted: 20 Apr 2021 Published: 17 Feb 2022

Academic Editor: Luca Quartuccio Copy Editor: Yue-Yue Zhang Production Editor: Yue-Yue Zhang

Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a distinct group of systemic
vasculitis with severe multi-organ involvement. It includes three types: granulomatosis with polyangiitis (GPA),
eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Cutaneous
manifestations are present at the time of presentation in around 35% of cases and are more frequently
encountered in EGPA than in GPA or MPA. They can also occur during the course of the disease or mark a disease
relapse. They can be classified into specific or non-specific according to the presence or absence of vasculitis,
whether or not associated with granuloma, in skin biopsies. The most common presentation of vasculitic skin
lesions in AAV is palpable purpura. Other manifestations include hemorrhagic blisters, tender subcutaneous
nodules, livedo reticularis/racemosa, painful ulcers that may mimic pyoderma gangrenosum (PG), and digital
gangrenes. A wide range of non-specific cutaneous manifestations can occur in association with AAV including
urticarial rash, prurigo, sterile pustules, and oral lesions. The presence of cutaneous lesions is usually associated
with severe organ involvement and systemic manifestations. Since skin lesions in AAV can occur in other types of
vasculitis and in other disorders, attributing them to AAV requires meticulous clinical, laboratory, and serological
correlation. Awareness of cutaneous lesions of AAV is important for all physicians working in the field of systemic
vasculitis as a skin biopsy may provide an easy clue to diagnose AAV in such cases.

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