Vasculitis Relapse is the Most Likely Diagnosis
This presentation of skin bleeding with reddish marks over both legs in a patient with lupus-ANCA vasculitis overlap on subtherapeutic immunosuppression strongly suggests active cutaneous vasculitis requiring immediate escalation of therapy. Your decision to increase prednisone to 30 mg daily is appropriate as an initial step.
Why This is Vasculitis, Not Steroid-Induced Purpura
Clinical Presentation Favors Vasculitis
- Skin is a commonly involved organ system in ANCA-associated vasculitis, presenting as palpable purpura or infiltrated erythema indicating dermal small-vessel vasculitis 1, 2, 3
- Cutaneous manifestations can appear as the first sign of relapse in ANCA vasculitis and may coexist with other organ involvement 4
- The bilateral lower extremity distribution is classic for vasculitic involvement 5, 3
Steroid-Induced Purpura is Unlikely at This Dose
- While corticosteroids can cause skin fragility and purpura, this typically occurs with prolonged high-dose therapy (not 5 mg maintenance dosing) and presents as painless, spontaneous hemorrhage with skin tearing—not the infiltrated erythematous lesions typical of vasculitis 6
- The FDA label confirms that corticosteroid-associated skin complications are dose-dependent 7
- 5 mg prednisolone is within the recommended maintenance dose range for AAV in remission and should not cause significant steroid-induced purpura 1, 2
Critical Problem: Subtherapeutic Immunosuppression
Your Patient's MMF Dose is Inadequate
- The KDIGO 2024 guidelines recommend MMF 2000 mg/day in divided doses for AAV maintenance therapy 1, 2
- Your patient is on only 500 mg twice daily (1000 mg/day)—half the recommended dose
- Subtherapeutic immunosuppression is a major relapse risk factor and substantially increases relapse risk 2
Immediate Diagnostic Workup Required
Before assuming this is isolated cutaneous vasculitis, you must evaluate for systemic involvement:
Laboratory Assessment
- ANCA titers (MPO and PR3) to evaluate serologic activity 2
- Urinalysis with microscopy to look for signs of renal relapse (hematuria, RBC casts, proteinuria) 2
- Serum creatinine to assess renal function 2
- Inflammatory markers (CRP, ESR) to assess systemic activity 2
Tissue Diagnosis
- Obtain a deep skin biopsy reaching the medium-sized vessels of the dermis (not a superficial punch biopsy) to confirm vasculitis 1, 3
- Consider direct immunofluorescence to distinguish IgA-associated vasculitis from IgG/IgM-associated vasculitis, which has prognostic significance 3
Treatment Algorithm
Immediate Management (Already Initiated)
- Increasing glucocorticoids (oral or intravenous) for acute relapse is appropriate 1, 2
- Your dose of prednisone 30 mg daily is reasonable as initial therapy for cutaneous vasculitis 5, 3
Optimize Maintenance Immunosuppression
- Increase MMF to 2000 mg/day in divided doses (1000 mg twice daily) to minimize relapse risk 1, 2
- This is the evidence-based maintenance dose for AAV 1
Consider Reinduction Therapy if Severe
- If vasculitis is confirmed with systemic features or extensive cutaneous involvement, consider reinduction therapy with rituximab 1, 2
- Patients with relapsing disease should be reinduced according to initial therapy guidelines, preferably with rituximab 1
Duration of Increased Glucocorticoids
- After achieving remission, glucocorticoids should be continued at 5-7.5 mg/day for 2 years, then slowly reduced by 1 mg every 2 months 1
Critical Pitfalls to Avoid
Do Not Wait for Biopsy Results
- If clinical presentation strongly suggests vasculitis with systemic features, start treatment immediately—do not wait for biopsy confirmation 2
- Delay in treatment can lead to irreversible organ damage
Do Not Continue Subtherapeutic MMF Dosing
- Continuing MMF at 1000 mg/day will perpetuate high relapse risk 2
- This is likely why your patient relapsed despite being on "maintenance therapy"
Monitor for Systemic Involvement
- Even if this appears to be isolated cutaneous vasculitis now, serial monitoring for renal, pulmonary, and neurologic involvement is mandatory 1, 2
- Cutaneous vasculitis can herald systemic relapse 4
Adjust MMF Dosing if Renal Function Declines
- If your patient develops significant renal insufficiency, MMF may require dose adjustment or closer monitoring for side effects 8