What is the recommended treatment for Henoch-Schönlein Purpura (HSP) rash?

Treatment of Henoch-Schönlein Purpura (HSP) Rash

Prednisone at 1 mg/kg/day for 2 weeks with tapering over the subsequent 2 weeks is the recommended treatment for Henoch-Schönlein Purpura rash, especially when accompanied by disturbing symptoms such as joint pain or abdominal pain.

First-Line Treatment for HSP Rash

Corticosteroid Therapy

• Prednisone is effective in treating HSP rash and associated symptoms:
  • Dosage: 1 mg/kg/day for 2 weeks, with tapering over the next 2 weeks 1
  • Significantly reduces the intensity of associated symptoms:
    • Abdominal pain (pain score 2.5 vs 4.8, p=0.029)
    • Joint pain (pain score 4.6 vs 7.3, p=0.030) 1
  • While prednisone doesn't prevent renal involvement, it effectively treats existing renal symptoms (61% resolution vs 34% with placebo) 1

When to Use Corticosteroids

• Indicated for HSP patients with:
  • Persistent or severe purpuric rash
  • Significant joint pain
  • Moderate to severe abdominal pain
  • Gastrointestinal bleeding
  • Early signs of renal involvement 1, 2

Second-Line Treatments

Dapsone for Chronic Cutaneous Manifestations

• Consider dapsone when cutaneous manifestations persist beyond 6 weeks:
  • Dosage: 1-2 mg/kg/day 3
  • Duration: Initially for 1 week, with extension based on response
  • Particularly effective for persistent purpuric lesions 3
  • Monitor for methemoglobinemia as a potential complication 3
  • Be aware that relapse may occur after treatment discontinuation (approximately 50% of cases) 3

Immunosuppressive Therapy for Severe Cases

• For severe or recurrent HSP with significant organ involvement:
  • Mycophenolate mofetil has shown effectiveness in cases with severe gastrointestinal involvement that are poorly responsive to steroids 4
  • High-dose intravenous pulse methylprednisolone coupled with azathioprine or cyclophosphamide may be beneficial in severe nephritis 2

Monitoring and Follow-up

During Treatment

• Monitor for:
  • Resolution of cutaneous lesions
  • Improvement in joint pain and abdominal symptoms
  • Urinalysis to detect renal involvement
  • Side effects of corticosteroid therapy

After Treatment

• Regular follow-up for at least 6 months
• Monitor for recurrences (approximately one-third of patients experience recurrence) 2
• Assess renal function, as long-term prognosis depends on severity of renal involvement 2

Important Considerations

• HSP is typically self-limiting but can have significant morbidity during the acute phase
• Early intervention with corticosteroids can reduce symptom severity and duration
• The general use of corticosteroids for all HSP cases is not supported, but patients with disturbing symptoms clearly benefit from early treatment 1
• Standard-dose corticosteroids have not been shown to prevent the development of nephritis 5
• Long-term prognosis is directly related to the severity of renal involvement 2

Remember that while HSP typically resolves spontaneously, prompt treatment of symptomatic cases with corticosteroids can significantly improve quality of life and potentially reduce complications during the acute phase.