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Cutaneous Vasculitis — Classification and Diagnostic Approach

Comprehensive 2012 Chapel Hill Consensus Conference (CHCC) classification of cutaneous vasculitides by vessel size — small (cutaneous leukocytoclastic angiitis, IgA vasculitis/Henoch-Schönlein purpura, urticarial vasculitis, ANCA-associated, cryoglobulinemic), medium (polyarteritis nodosa cutaneous variant, Kawasaki, Buerger), large (giant cell arteritis, Takayasu); diagnostic algorithm includes skin biopsy with H&E and direct immunofluorescence, ANCA, complement, cryoglobulins, hepatitis serology, and systemic workup; treatment ranges from topical and supportive care to systemic immunosuppression.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is Cutaneous Vasculitis — Classification and Diagnostic Approach?

Cutaneous vasculitis represents inflammation and damage of blood vessels of the skin, manifesting clinically as palpable purpura (hallmark), petechiae, urticarial lesions persisting >24 hours, pustules, papules, nodules, livedo reticularis, ulcers, gangrene, and digital ischemia depending on vessel size and severity. Cutaneous involvement may be the sole manifestation or part of systemic vasculitis with multiorgan involvement requiring urgent diagnosis and treatment.

The 2012 Chapel Hill Consensus Conference (CHCC) classification provides standardized nomenclature and definitions, organizing vasculitides primarily by predominant vessel size affected, with additional categories for variable vessel involvement, single organ vasculitis, and vasculitis associated with systemic disease or probable etiology. This replaces older classification systems (1990 ACR criteria) and incorporates current pathophysiologic understanding.

Small vessel vasculitis (predominantly arterioles, venules, and capillaries): includes cutaneous leukocytoclastic angiitis (CLA — idiopathic skin-limited LCV, most common form), IgA vasculitis (Henoch-Schönlein purpura — characteristic tetrad of palpable purpura, abdominal pain, arthralgia, glomerulonephritis with IgA1 immune deposits), cryoglobulinemic vasculitis (mixed type II/III, often associated with hepatitis C), hypocomplementemic urticarial vasculitis (HUV — McDuffie syndrome with anti-C1q antibody), ANCA-associated vasculitis (AAV — three subtypes: granulomatosis with polyangiitis [GPA, formerly Wegener with c-ANCA/PR3], microscopic polyangiitis [MPA with p-ANCA/MPO], eosinophilic granulomatosis with polyangiitis [EGPA, formerly Churg-Strauss]).

Medium vessel vasculitis (predominantly muscular arteries): polyarteritis nodosa (PAN — systemic form with subcutaneous nodules, livedo, ulcers, mononeuritis multiplex, renal and gastrointestinal involvement; cutaneous PAN limited to skin with subcutaneous nodules and livedo, often more chronic relapsing), Kawasaki disease (childhood vasculitis with coronary aneurysm risk), Buerger disease (thromboangiitis obliterans — distal extremity vasculitis primarily in young male tobacco users, ischemia and gangrene). Large vessel vasculitis (aorta and major branches): giant cell arteritis (temporal arteritis — affects elderly with temporal artery tenderness, jaw claudication, vision loss [risk of permanent blindness], polymyalgia rheumatica association, very high ESR/CRP), Takayasu arteritis (affects young women, especially Asian descent, aortic arch syndrome, pulseless extremity, hypertension). Variable vessel vasculitis: Behçet disease (Mediterranean and Asian populations, oral and genital aphthous ulcers, uveitis, pathergy, HLA-B51 association), Cogan syndrome (interstitial keratitis with audiovestibular dysfunction).

Symptoms

Palpable purpura — hallmark cutaneous lesion (raised, non-blanching purple papules)
Petechiae and macular purpura (small vessel involvement)
Urticarial lesions persisting >24 hours with bruising on resolution (urticarial vasculitis)
Subcutaneous nodules along course of artery (medium vessel — PAN, EGPA)
Livedo reticularis (mottled purple-blue net pattern — medium vessel)
Ulcers, gangrene, digital ischemia (severe medium-large vessel)
Pustules, papules, vesicles (less common forms)
Distribution: lower extremities most common (gravity-dependent)
Systemic features: fever, malaise, joint pain, abdominal pain, hematuria, peripheral neuropathy
Pulmonary symptoms (cough, hemoptysis — AAV)
Sinus and upper respiratory symptoms (GPA — saddle nose, otitis)
Vision loss or jaw claudication (GCA — emergent)

Risk Factors

Recent infection (streptococcus, hepatitis B/C, HIV, parvovirus B19, COVID-19)
Drug exposure (propylthiouracil, hydralazine, minocycline, allopurinol, antibiotics)
Hepatitis C infection (cryoglobulinemic vasculitis)
Connective tissue disease (SLE, RA, Sjögren)
Malignancy (paraneoplastic vasculitis)
Levamisole-contaminated cocaine (ANCA-positive vasculitis with retiform purpura)
Tobacco use (Buerger disease)
Family history (Behçet — HLA-B51)
Age and sex (GCA elderly, Takayasu young women, Kawasaki children, Buerger young male smokers)
Geographic and ethnic factors (Behçet Mediterranean/Asian, Takayasu Asian)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Sudden onset of palpable purpura especially with systemic symptoms
  • Vasculitic rash with abdominal pain, joint pain, or hematuria (Henoch-Schönlein in child)
  • Persistent urticarial lesions (>24 hours) with bruising on resolution
  • Vision loss, headache, or jaw claudication in elderly (GCA emergency)
  • Cough, hemoptysis, sinus symptoms with cutaneous vasculitis (AAV — emergent)
  • Mononeuritis multiplex (foot drop, wrist drop) with systemic vasculitis features
  • Distal extremity ischemia or gangrene in young smoker (Buerger)
  • Cutaneous nodules along arterial course
  • Recent drug exposure with new vasculitis
  • Recurrent oral and genital ulcers with eye involvement (Behçet)

Treatment Methods

01
Diagnostic workup: comprehensive history (drug exposure including OTC, herbal, infection, recent vaccines, fever, weight loss, fatigue, joint pain, abdominal pain, hematuria, neurologic deficits, pulmonary symptoms), thorough physical examination (skin morphology and distribution, oral and genital mucosa, peripheral pulses, neurologic, ENT in suspected GPA, ophthalmologic in GCA-Behçet)
02
Skin biopsy: punch biopsy 4–6 mm of fresh lesion <24–48 hours old (palpable purpura preferred over older purpura), include subcutis if medium vessel suspected, request histopathology with H&E (leukocytoclastic vasculitis pattern — fibrinoid necrosis of small vessels with fragmented PMNs and karyorrhexis is small vessel hallmark; medium vessel shows segmental fibrinoid necrosis with mixed inflammation), and immunofluorescence on second fresh biopsy (IgA dominant — IgA vasculitis; IgM/C3 — cryoglobulinemic; C1q — HUV; pauci-immune with no significant immune deposits — ANCA-associated)
03
Laboratory workup (small vessel vasculitis): CBC with differential (eosinophilia in EGPA, leukopenia in SLE-vasculitis), comprehensive metabolic panel, urinalysis with microscopy and protein-creatinine ratio (microscopic hematuria, proteinuria, RBC casts indicate glomerulonephritis), ESR and CRP, ANA and ENA panel (anti-Ro, La, Sm, RNP), ANCA (c-ANCA/PR3 — GPA, p-ANCA/MPO — MPA, EGPA), complement C3/C4 (low in immune complex disease — SLE, cryoglobulinemic, HUV), cryoglobulins (collected and processed at 37°C — important for handling), rheumatoid factor (often elevated in cryoglobulinemic), serum protein electrophoresis and immunofixation, hepatitis B serology (HBsAg, anti-HBc, anti-HBs), hepatitis C antibody and PCR, HIV test, RPR, blood cultures if febrile, ASO and anti-DNase B if post-streptococcal suspected
04
Imaging: chest X-ray (cavitary lesions in GPA, infiltrates), chest CT (high-resolution if suspected ANCA-associated), sinus CT in GPA, abdominal CT-angiography if PAN suspected, MR-angiography or conventional angiography for large vessel disease, echocardiography in EGPA
05
Additional biopsies: kidney biopsy if glomerulonephritis (light microscopy, immunofluorescence, electron microscopy — pauci-immune crescentic in AAV, IgA dominant in IgA vasculitis), lung biopsy (rarely needed; characteristic findings in AAV), nerve biopsy in mononeuritis (axonal injury with vasculitis)
06
Treatment by type: (1) Idiopathic skin-limited cutaneous LCV — supportive (leg elevation, compression, NSAIDs for arthralgia, topical corticosteroids if pruritic, wound care for ulcers), most resolve in 4–6 weeks; (2) IgA vasculitis (Henoch-Schönlein) — usually self-limited in children with supportive care, oral corticosteroids for severe abdominal pain or arthritis, careful monitoring of renal function, immunosuppression for severe nephritis; (3) Cryoglobulinemic vasculitis — treat underlying hepatitis C with direct-acting antivirals (sofosbuvir-velpatasvir or similar — often clears vasculitis), rituximab for severe disease, plasmapheresis for fulminant; (4) Hypocomplementemic urticarial vasculitis — antihistamines, dapsone, hydroxychloroquine, oral corticosteroids; (5) ANCA-associated vasculitis — induction with rituximab or cyclophosphamide plus high-dose corticosteroids, plasmapheresis for severe rapidly progressive disease or alveolar hemorrhage, maintenance with rituximab or methotrexate-azathioprine, avacopan as steroid-sparing agent; (6) Polyarteritis nodosa — corticosteroids plus cyclophosphamide for severe systemic, antiviral therapy for hepatitis B-associated; (7) Giant cell arteritis — high-dose oral corticosteroids (prednisone 60 mg/day, IV methylprednisolone 1 g/day x3 if vision loss), tocilizumab as steroid-sparing; (8) Takayasu — corticosteroids plus methotrexate or biologics; (9) Behçet — colchicine for mucocutaneous, corticosteroids and azathioprine for organ involvement, biologics (TNF inhibitors, apremilast, IL-1 antagonists, IL-6 antagonists)
07
Drug-induced vasculitis: discontinue offending drug (often resolves in weeks), supportive care, occasional brief corticosteroid course if severe; documented for future avoidance
08
Long-term: regular follow-up depending on type and severity, monitoring of disease activity (BVAS — Birmingham Vasculitis Activity Score), surveillance for relapse, organ damage (VDI — Vasculitis Damage Index), cardiovascular risk reduction in chronic disease, vaccination adherence (live vaccines contraindicated on immunosuppression), pregnancy planning with appropriate medication adjustment, multidisciplinary care for complex disease (rheumatology, dermatology, nephrology, pulmonology, infectious disease, ophthalmology)

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