Treatment of Leukocytoclastic Vasculitis with IgA-Positive Skin Biopsy
For leukocytoclastic vasculitis with IgA deposition (Henoch-Schönlein purpura), treatment should be conservative for isolated cutaneous disease, but systemic corticosteroids are indicated for organ-threatening manifestations, particularly renal involvement. 1
Disease Severity Assessment
The presence of IgA on immunofluorescence indicates Henoch-Schönlein purpura (HSP), which carries a higher risk of systemic involvement and worse prognosis compared to IgG/IgM-mediated leukocytoclastic vasculitis. 1, 2 This distinction is critical because:
- IgA-associated vasculitis has greater likelihood of systemic involvement in both children and adults 1
- Adults with HSP experience more severe systemic disease than children 2
- IgG/IgM-mediated vasculitis has better prognosis and less systemic involvement 1, 3
Mandatory Initial Evaluation
Before initiating treatment, assess for:
- Renal involvement: Urinalysis for microscopic hematuria, red cell casts, and proteinuria (1-3 g/day suggests significant glomerulonephritis) 4
- Gastrointestinal involvement: Abdominal pain, gastrointestinal bleeding 1
- Joint involvement: Arthralgias or arthritis 1
- Skin necrosis: Hemorrhagic blisters indicating incipient necrosis 3, 2
Treatment Algorithm by Disease Severity
Mild Cutaneous Disease Only
For isolated palpable purpura without systemic involvement:
- Leg elevation and compression stockings to reduce purpura 1
- Avoidance of prolonged standing 5
- NSAIDs for symptomatic relief 5
- Identify and eliminate triggers: Review medications, treat infections, obtain throat swabs 1
Most cases are self-limited and resolve within weeks with conservative management alone. 6
Moderate Disease: Chronic or Relapsing Cutaneous Vasculitis
When purpura persists or recurs despite conservative measures:
- First-line: Colchicine 1, 3, 5
- Second-line: Dapsone (requires G6PD screening and monitoring for blood dyscrasias, neuropathy, and hypersensitivity syndrome) 1, 3, 5
- Alternative: Topical dapsone may provide efficacy with fewer adverse effects in selected patients 6
Severe Cutaneous Disease
When hemorrhagic blisters or signs of incipient skin necrosis appear:
- Systemic corticosteroids are indicated immediately to prevent progression to ulceration 1, 3, 2
- Typical dosing: Prednisone 1 mg/kg/day (maximum 80 mg daily) 7
Severe Systemic Disease
For organ-threatening manifestations (severe glomerulonephritis, pulmonary hemorrhage, rapidly progressive renal failure):
- Combination therapy with glucocorticoids PLUS cyclophosphamide or rituximab 7
- Consider plasma exchange for rapidly progressive renal failure 7
- ACE inhibitors may be helpful for symptomatic management of glomerulonephritis 3
Critical Caveats
Important limitation: In adults with HSP and severe glomerulonephritis, there is insufficient evidence that glucocorticoids reduce the incidence of severe renal insufficiency. 3 Some studies suggest benefit in children, but no studies demonstrate this effect in adults. 1 Despite this evidence gap, corticosteroids remain standard practice for severe systemic involvement given the high risk of permanent organ damage.
Avoid aggressive immunosuppression for isolated cutaneous disease, as the favorable natural course does not justify the toxicity risk. 1, 2
Monitor for progression: HSP can involve multiple organs over time, requiring serial assessment of renal function and systemic symptoms even after initial presentation. 1