Cutaneous Vasculitis: Diagnosis, Management, Key Insights
Comprehensive CME on systemic and cutaneous vasculitis: clinical features, diagnosis, and management strategies for dermatologists.
Author: Dr Delwyn Dyall-Smith, Dermatologist, Australia, 2010 (updated 2023).
Cutaneous vasculitis refers to inflammation of small or medium-sized blood vessels in the skin, often presenting as palpable purpura, but can include a range of lesions such as urticaria, nodules, ulcers, and livedo reticularis. When associated with systemic vasculitis, skin findings provide crucial diagnostic clues, requiring prompt evaluation for organ involvement. This CME article examines key systemic vasculitides with prominent cutaneous manifestations, emphasising clinical recognition, biopsy techniques, and management principles grounded in recent guidelines.
What is cutaneous vasculitis?
Cutaneous vasculitis involves leukocytoclastic vasculitis (LCV) of post-capillary venules or deeper vessel inflammation in medium-vessel vasculitides. Skin-limited forms resolve spontaneously in 90% of cases within 3-4 weeks, but systemic associations demand investigation. Lesions typically arise in crops over days, lasting up to 3 weeks per episode, with potential for scarring if ulcerative.
Who gets cutaneous vasculitis?
Systemic vasculitides with cutaneous involvement affect all ages, though IgA vasculitis predominates in children, while ANCA-associated forms increase in adults over 50. Risk factors include infections, drugs, autoimmune diseases, and malignancies. Females are disproportionately affected in conditions like eosinophilic granulomatosis with polyangiitis (EGPA).
Clinical features of cutaneous vasculitis in systemic disease
Skin lesions vary by vessel size and underlying pathology:
- Small-vessel vasculitis: Palpable purpura on lower legs, evolving to vesicles, bullae, or necrosis. Urticarial lesions suggest hypocomplementaemic forms.
- Medium-vessel vasculitis: Subcutaneous nodules, livedo reticularis, or digital infarcts.
- Systemic signs: Fever, arthralgias, renal involvement (haematuria), neuropathy, or pulmonary symptoms guide urgency.
IgA vasculitis (Henoch–Schönlein purpura)
The most common systemic vasculitis in children, presenting with palpable purpura on lower extremities, often triggered by upper respiratory infections. Associated with abdominal pain, arthritis, and IgA nephropathy (frank haematuria or renal impairment in 50%). Skin biopsy shows leukocytoclastic vasculitis with IgA deposits on immunofluorescence. Most cases self-limit in 4 weeks, but monitor renal function long-term.
ANCA-associated small vessel vasculitis
Encompasses granulomatosis with polyangiitis (GPA, anti-PR3), microscopic polyangiitis (MPA, anti-MPO), and eosinophilic granulomatosis with polyangiitis (EGPA, anti-MPO/p-ANCA). Cutaneous features include palpable purpura (60-70%), oral ulcers, and splinter haemorrhages. GPA often shows necrotic ulcers on face/ears; EGPA features palpable purpura with asthma, eosinophilia, neuropathy. Urgent assessment for renal/pulmonary involvement essential.
Cryoglobulinaemia
Mixed cryoglobulinaemia (type II/III) causes purpura, arthralgias, weakness, and glomerulonephritis, often linked to hepatitis C. Skin findings: retiform purpura, livedo, ulcers on acral sites. Serum cryoglobulins and low C4 confirm diagnosis. Skin-limited forms respond to colchicine/dapsone.
Drug-induced vasculitis
Common triggers: antibiotics, NSAIDs, biologics. Presents as leukocytoclastic vasculitis 7-21 days post-exposure. Resolution follows drug cessation; biopsy aids identification.
Other systemic vasculitides
- Behçet disease: Oral/genital ulcers, erythema nodosum, pathergy.
- Polyarteritis nodosa (PAN): Tender subcutaneous nodules along vessels, livedo, ulcers on calves.
- Sweet syndrome: Painful plaques with neutrophilic infiltrate, associated with malignancy.
Diagnosis of cutaneous vasculitis
Diagnosis integrates history, exam, labs, and biopsy:
- History: Drugs, infections, systemic symptoms, travel.
- Exam: Lesion morphology, distribution, extracutaneous signs.
- Labs: CBC, ESR/CRP, urinalysis, ANCA, ANA, cryoglobulins, hepatitis serologies.
- Biopsy: Punch biopsy of early lesion (<48h) for H&E and direct immunofluorescence (DIF). DIF shows IgA in Henoch-Schönlein, fibrinogen in PAN.
| Vasculitis Type | Key Histology | DIF Findings |
|---|---|---|
| IgA vasculitis | LCV post-capillary venules | IgA dominant |
| ANCA vasculitis | LCV ± granulomatous | Fibrinogen |
| Cryoglobulinaemia | LCV with hyaline thrombi | C3, IgM/IgG |
| PAN | Medium vessel fibrinoid necrosis | Fibrinogen |
Differential diagnosis
- Infections: Rickettsia, meningococcaemia, embolic phenomena.
- Thrombotic: Antiphospholipid syndrome, cryofibrinogenaemia.
- Non-inflammatory: Pigmented purpuric dermatoses, venous stasis.
Investigations for systemic vasculitis
Screen for organ involvement:
- Urinalysis for glomerular disease.
- Chest X-ray/CT for alveolar haemorrhage.
- ANCA, complement, rheumatoid factor.
- Biopsy of affected organs if indicated.
Management of cutaneous vasculitis
Treatment escalates by severity and systemic involvement:
| Scenario | Treatment Ladder |
|---|---|
| Skin-limited (90% cases) | Remove triggers, rest/elevation, NSAIDs, topical steroids. Colchicine/dapsone if persistent. |
| Symptomatic/ulcerative | Prednisone 0.5-1mg/kg/day, taper over 3-6 weeks. |
| Refractory hypocomplementaemic | Hydroxychloroquine, MMF, rituximab. |
| Systemic (e.g., ANCA) | High-dose steroids + cyclophosphamide/rituximab; plasma exchange for severe renal. |
Supportive: Leg elevation, compression, wound care to prevent ulcers/infection.
Relapse of cutaneous vasculitis
Recurrences common (up to 50%), often milder. Investigate new triggers; prognosis excellent for skin-limited disease.
Untreated cutaneous vasculitis
Most self-resolve, but systemic forms risk renal failure, neuropathy, or mortality if untreated.
Prevention of cutaneous vasculitis
Avoid known drugs/infections; vaccinate against triggers like streptococcus.
Systemic vasculitis prognosis
Variable: IgA vasculitis >90% renal recovery; ANCA 5-year survival 70-80% with treatment.
Frequently asked questions
What does cutaneous vasculitis look like?
Palpable purpura on legs, progressing to blisters/ulcers; medium vessel shows nodules/livedo.
How is cutaneous vasculitis diagnosed?
Clinical + biopsy with DIF; labs rule out systemic disease.
What is the treatment for cutaneous vasculitis?
Treat cause; supportive for mild, immunosuppressants for severe/systemic.
What is the cause of cutaneous vasculitis?
Idiopathic, drugs, infections, autoimmune, paraproteinaemia.
Can cutaneous vasculitis be cured?
Skin-limited resolves; systemic requires chronic management.
References
- Treatment of cutaneous vasculitis — PMC (NCBI). 2022-12-15. https://pmc.ncbi.nlm.nih.gov/articles/PMC9716566/
- Cutaneous Small-Vessel Vasculitis — Vasculitis Foundation. 2023-05-10. https://vasculitisfoundation.org/education/vasculitis-types/cutaneous-small-vessel-vasculitis/
- Cutaneous Vasculitis – Symptoms, Causes, Treatment — NORD (rarediseases.org). 2024-01-22. https://rarediseases.org/rare-diseases/cutaneous-vasculitis/
- Cutaneous vasculitis — DermNet NZ. 2023-11-08. https://dermnetnz.org/topics/cutaneous-vasculitis
- Cutaneous Vasculitis Information for Patients — Rheumatology Dermatology Society. 2022-07-14. https://www.rheumaderm-society.org/vasculitis-information-for-patients/
- Vasculitis – Information for Patients — American College of Rheumatology. 2023-09-19. https://rheumatology.org/patients/vasculitis
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