The clinical presentation of the various vasculitides on the skin and internal organs is mostly determined by the diameter or size of the vessels mainly affected.[{{cite book | last=Sunderkötter | first=Cord | title=Braun-Falco´s Dermatology | chapter=Vasculitis and Vasculopathies | publisher=Springer Berlin Heidelberg | publication-place=Berlin, Heidelberg | date=2022 | isbn=978-3-662-63708-1 | doi=10.1007/978-3-662-63709-8_64 | page=1125–1169}}] Non-specific symptoms are common and include fever, headache, fatigue, myalgia, weight loss, and arthralgia.[{{cite book |last1=Luqmani |first1=Raashid |last2=Robson |first2=Joanna |last3=Suppiah |first3=Ravi |editor1-last=Davey |editor1-first=Patrick |editor2-last=Sprigings |editor2-first=David |title=Diagnosis and Treatment in Internal Medicine |date=August 2018 |publisher=Oxford University Press |isbn=978-0-19-956874-1 |pages=927-931 |url=https://books.google.ca/books/about/Diagnosis_and_Treatment_in_Internal_Medi.html?id=hWFmDwAAQBAJ&redir_esc=y |access-date=8 August 2024 |chapter=272: Vasculitis}}][{{cite journal | last=Jayne | first=David | title=The diagnosis of vasculitis | journal=Best Practice & Research Clinical Rheumatology | volume=23 | issue=3 | date=2009 | doi=10.1016/j.berh.2009.03.001 | pages=445–453}}]
All forms of vasculitis, even large vessel vasculitides, may cause skin manifestations. The most common skin manifestations include purpura, nodules, livedo reticularis, skin ulcers, and purpuric urticaria.[{{cite journal | last=Shavit | first=Eran | last2=Alavi | first2=Afsaneh | last3=Sibbald | first3=R. Gary | title=Vasculitis—What Do We Have to Know? A Review of Literature | journal=The International Journal of Lower Extremity Wounds | volume=17 | issue=4 | date=2018 | issn=1534-7346 | doi=10.1177/1534734618804982 | pages=218–226}}]
| class="wikitable"
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!Type
!Name
!Main symptoms |
rowspan="2" |Primary large vessel vasculitis[{{cite journal |last=Jennette |first=J. Charles |date=27 September 2013 |title=Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides |journal=Clinical and Experimental Nephrology |publisher=Springer Science and Business Media LLC |volume=17 |issue=5 |pages=603–606 |doi=10.1007/s10157-013-0869-6 |issn=1342-1751 |pmc=4029362 |pmid=24072416}}]
|Takayasu arteritis
|Diminished or absent pulses, vascular bruits, hypertension, Takayasu retinopathy, and aortic regurgitation.[{{cite journal | last=Johnston | first=S L | last2=Lock | first2=R J | last3=Gompels | first3=M M | title=Takayasu arteritis: a review | journal=Journal of Clinical Pathology | publisher=BMJ Publishing Group | volume=55 | issue=7 | date=2024-03-14 | pmid=12101189 | doi=10.1136/jcp.55.7.481 | url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769710/ | access-date=2024-08-18 | page=}}] |
| Giant cell arteritis
|Headache, scalp tenderness, jaw claudication, and blindness.[{{cite journal | last=Hoffman | first=Gary S. | title=Giant Cell Arteritis | journal=Annals of Internal Medicine | volume=165 | issue=9 | date=2016-11-01 | issn=0003-4819 | doi=10.7326/AITC201611010 | page=ITC67}}] |
rowspan="2" |Primary medium vessel vasculitis|Polyarteritis nodosa
|Mononeuritis multiplex, nodules, purpura, livedo, and hypertension.[{{cite journal | last=Forbess | first=Lindsy | last2=Bannykh | first2=Serguei | title=Polyarteritis Nodosa | journal=Rheumatic Disease Clinics of North America | volume=41 | issue=1 | date=2015 | doi=10.1016/j.rdc.2014.09.005 | pages=33–46}}] |
| Kawasaki disease
|Fever, conjunctivitis, exanthema, palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema.[{{cite journal | last=Son | first=M. B. F. | last2=Newburger | first2=J. W. | title=Kawasaki Disease | journal=Pediatrics in Review | volume=34 | issue=4 | date=2013-04-01 | issn=0191-9601 | doi=10.1542/pir.34-4-151 | pages=151–162}}][{{cite journal | last=Hedrich | first=Christian M. | last2=Schnabel | first2=Anja | last3=Hospach | first3=Toni | title=Kawasaki Disease | journal=Frontiers in Pediatrics | volume=6 | date=2018-07-10 | issn=2296-2360 | pmid=30042935 | pmc=6048561 | doi=10.3389/fped.2018.00198 | doi-access=free | page=}}] |
rowspan="3" |Primary small vessel antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis|Microscopic polyangiitis
|Focal segmental rapidly progressive glomerulonephritis, proteinuria, hemoptysis, palpable purpura, abdominal pain, and peripheral neuropathy.[{{cite journal | last=Chung | first=Sharon A. | last2=Seo | first2=Philip | title=Microscopic Polyangiitis | journal=Rheumatic Disease Clinics of North America | volume=36 | issue=3 | date=2010 | pmid=20688249 | pmc=2917831 | doi=10.1016/j.rdc.2010.04.003 | pages=545–558}}] |
| Granulomatosis with polyangiitis
|Crusting rhinorrhea, sinusitis, chronic otitis
media, nasal obstruction, shortness of breath, and chronic cough.[{{cite journal | last=Comarmond | first=Cloé | last2=Cacoub | first2=Patrice | title=Granulomatosis with polyangiitis (Wegener): Clinical aspects and treatment | journal=Autoimmunity Reviews | volume=13 | issue=11 | date=2014 | doi=10.1016/j.autrev.2014.08.017 | pages=1121–1125}}][{{cite journal | last=Grygiel-Górniak | first=Bogna | last2=Limphaibool | first2=Nattakarn | last3=Perkowska | first3=Katarzyna | last4=Puszczewicz | first4=Mariusz | title=Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features | journal=Postgraduate Medicine | volume=130 | issue=7 | date=2018-10-03 | issn=0032-5481 | doi=10.1080/00325481.2018.1503920 | pages=581–596}}] |
| Eosinophilic granulomatosis with polyangiitis
|Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain.[{{cite journal | last=Vaglio | first=A. | last2=Buzio | first2=C. | last3=Zwerina | first3=J. | title=Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): state of the art | journal=Allergy | volume=68 | issue=3 | date=2013 | issn=0105-4538 | doi=10.1111/all.12088 | pages=261–273}}][{{cite journal | last=White | first=Jpe | last2=Dubey | first2=S. | title=Eosinophilic granulomatosis with polyangiitis: A review | journal=Autoimmunity Reviews | volume=22 | issue=1 | date=2023 | doi=10.1016/j.autrev.2022.103219 | page=103219}}] |
rowspan="4" |Primary immune complex small vessel vasculitis|Anti-glomerular basement membrane disease
|Glomerulonephritis, lung hemorrhage, hematuria, hemoptysis, cough, and dyspnea.[{{cite journal | last=Bharati | first=Joyita | last2=Jhaveri | first2=Kenar D. | last3=Salama | first3=Alan D. | last4=Oni | first4=Louise | title=Anti–Glomerular Basement Membrane Disease: Recent Updates | journal=Advances in Kidney Disease and Health | volume=31 | issue=3 | date=2024 | doi=10.1053/j.akdh.2024.04.007 | pages=206–215}}] |
| Cryoglobulinemic vasculitis
|Palpable purpura, Raynaud's phenomenon, joint pain,[{{cite journal | last=Silva | first=Filipa | last2=Pinto | first2=Claudemira | last3=Barbosa | first3=Arsénio | last4=Borges | first4=Tiago | last5=Dias | first5=Carlos | last6=Almeida | first6=Jorge | title=New insights in cryoglobulinemic vasculitis | journal=Journal of Autoimmunity | volume=105 | date=2019 | doi=10.1016/j.jaut.2019.102313 | page=102313}}] and peripheral neuropathy. |
| IgA vasculitis
|Palpable purpura, arthralgia, abdominal pain, nephritis, and haematuria.[{{cite journal | last=Pillebout | first=Evangéline | last2=Sunderkötter | first2=Cord | title=IgA vasculitis | journal=Seminars in Immunopathology | volume=43 | issue=5 | date=2021 | issn=1863-2297 | doi=10.1007/s00281-021-00874-9 | pages=729–738}}] |
| Hypocomplementemic urticarial vasculitis
|Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease.[{{cite journal | last=Gu | first=Stephanie L. | last2=Jorizzo | first2=Joseph L. | title=Urticarial vasculitis | journal=International Journal of Women's Dermatology | volume=7 | issue=3 | date=2021 | pmid=34222586 | pmc=8243153 | doi=10.1016/j.ijwd.2021.01.021 | pages=290–297}}] |
rowspan="2" |Primary variable vessel vasculitis|Behcet’s disease
|Oral ulcers, genital ulcers, papulopustular lesions, uveitis, superficial venous thrombosis and deep vein thrombosis.[{{cite journal | last=Bettiol | first=Alessandra | last2=Prisco | first2=Domenico | last3=Emmi | first3=Giacomo | title=Behçet: the syndrome | journal=Rheumatology | volume=59 | issue=Supplement_3 | date=2020-05-01 | issn=1462-0324 | doi=10.1093/rheumatology/kez626 | pages=iii101–iii107}}] |
| Cogan’s syndrome
|Interstitial keratitis, ocular redness, vertigo, and tinnitus.[{{cite journal | last=Iliescu | first=Daniela Adriana | last2=Timaru | first2=Cristina Mihaela | last3=Batras | first3=Mehdi | last4=Simone | first4=Algerino De | last5=Stefan | first5=Cornel | title=COGAN’S SYNDROME | journal=Romanian Journal of Ophthalmology | publisher=Romanian Society of Ophthalmology | volume=59 | issue=1 | date=2024-03-14 | pmid=27373108 | url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5729811/ | access-date=2024-08-19 | page=}}] |
rowspan="4" |Single-organ vasculitis[{{cite journal |last=Martins-Martinho |first=Joana |last2=Dourado |first2=Eduardo |last3=Khmelinskii |first3=Nikita |last4=Espinosa |first4=Pablo |last5=Ponte |first5=Cristina |date=2021 |title=Localized Forms of Vasculitis |journal=Current Rheumatology Reports |volume=23 |issue=7 |page= |doi=10.1007/s11926-021-01012-y |issn=1523-3774 |pmc=8247627 |pmid=34196889}}]|Cutaneous small-vessel vasculitis
|Palpable purpura, necrosis, ulceration, bullae, and nodules.[{{cite journal | last=Russell | first=James P. | last2=Gibson | first2=Lawrence E. | title=Primary cutaneous small vessel vasculitis: approach to diagnosis and treatment | journal=International Journal of Dermatology | volume=45 | issue=1 | date=2006 | issn=0011-9059 | doi=10.1111/j.1365-4632.2005.02898.x | pages=3–13}}] |
| Cutaneous arteritis
|Nodules, livedo reticularis, ulcers, and gangrene.[{{cite journal | last=Furukawa | first=Fukumi | title=Cutaneous Polyarteritis Nodosa: An Update | journal=Annals of Vascular Diseases | publisher=Editorial Committee of Annals of Vascular Diseases | volume=5 | issue=3 | date=2012 | pmid=23555526 | doi=10.3400/avd.ra.12.00061 | url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3595843/ | access-date=2024-08-19 | page=}}] |
| Primary central nervous system vasculitis
|Headache, cognitive impairment, stroke, encephalopathy, and seizures.[{{cite journal | last=Junek | first=Mats | last2=Perera | first2=Kanjana S | last3=Kiczek | first3=Matthew | last4=Hajj-Ali | first4=Rula A | title=Current and future advances in practice: a practical approach to the diagnosis and management of primary central nervous system vasculitis | journal=Rheumatology Advances in Practice | volume=7 | issue=3 | date=2023-08-26 | issn=2514-1775 | pmid=38091383 | pmc=10712448 | doi=10.1093/rap/rkad080 | page=}}]
| |
| Retinal vasculitis
|Visual impairments, floaters, and macular edema.[{{cite journal | last=Abu El-Asrar | first=Ahmed M. | last2=Herbort | first2=Carl P. | last3=Tabbara | first3=Khalid F. | title=Retinal Vasculitis | journal=Ocular Immunology and Inflammation | volume=13 | issue=6 | date=2005 | issn=0927-3948 | doi=10.1080/09273940591003828 | pages=415–433}}] |
rowspan="2" |Secondary vasculitis|Lupus vasculitis
|Palpable purpura, petechiae, papulonodular lesions, urticaria lesions, and mononeuritis multiplex.[{{cite journal | last=Leone | first=Patrizia | last2=Prete | first2=Marcella | last3=Malerba | first3=Eleonora | last4=Bray | first4=Antonella | last5=Susca | first5=Nicola | last6=Ingravallo | first6=Giuseppe | last7=Racanelli | first7=Vito | title=Lupus Vasculitis: An Overview | journal=Biomedicines | publisher=MDPI AG | volume=9 | issue=11 | date=2021-11-05 | issn=2227-9059 | doi=10.3390/biomedicines9111626 | doi-access=free | page=1626}}] |
| Rheumatoid vasculitis
|Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex.[{{cite journal | last=Bartels | first=Christie M. | last2=Bridges | first2=Alan J. | title=Rheumatoid Vasculitis: Vanishing Menace or Target for New Treatments? | journal=Current Rheumatology Reports | publisher=Springer Science and Business Media LLC | volume=12 | issue=6 | date=2010-09-15 | issn=1523-3774 | doi=10.1007/s11926-010-0130-1 | pages=414–419}}]
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