What is the management approach for Henoch-Schönlein Purpura (HSP) rash?

Management of Henoch-Schönlein Purpura (HSP) Rash

For Henoch-Schönlein Purpura (HSP) rash, supportive care is the primary management approach, with monitoring for systemic complications and consideration of immunosuppressive therapy only for severe cases with organ involvement.

Clinical Presentation and Diagnosis

• HSP is characterized by a distinctive non-thrombocytopenic petechial or purpuric rash, typically on the lower extremities and buttocks
• The rash is often accompanied by:
  • Migratory polyarthralgias/arthritis (58.1% of cases) 1
  • Abdominal pain (56% of cases) 1
  • Subcutaneous edema (38.8% of cases) 1
  • Renal involvement (29.8% of cases) 1

Management of HSP Rash

First-line Management (Mild Cases)

• Supportive care:

  • Provide patient education about the typically self-limiting nature of the disease 2
  • Ensure adequate hydration
  • Apply moisturizers to affected skin areas
  • Avoid skin irritants and mechanical trauma to the rash

• Pain management:

  • NSAIDs (e.g., ibuprofen) for joint pain and mild skin discomfort
  • Avoid NSAIDs if significant renal involvement is present

Monitoring Requirements

• Regular urine testing for at least 6 months to detect proteinuria and hematuria 2
• Blood pressure monitoring at each follow-up visit
• More frequent monitoring for patients with:
  • Severe initial presentation
  • Persistent rash
  • Abdominal pain
  • Older age at onset 3

Management of Moderate to Severe Cases

• For persistent or extensive rash with significant discomfort:

  • Short course of oral corticosteroids (prednisone 1-2 mg/kg/day for 1-2 weeks with taper)
  • Consider topical high-potency steroids for localized areas of severe inflammation

• For recurrent rash or frequent relapses:

  • Consider colchicine, which has shown favorable response in relapsing cases unresponsive to ibuprofen or steroids 1

Management of Systemic Involvement

Gastrointestinal Involvement

• For severe abdominal pain:
  • Hospital admission for observation
  • IV hydration
  • Short course of systemic corticosteroids
  • Monitor for intussusception (increased risk with severe GI involvement) 1

Renal Involvement

• For patients with significant proteinuria, hematuria, or declining renal function:
  • Nephrology consultation
  • Consider immunosuppressive therapy for biopsy-proven nephritis
  • Mycophenolate mofetil has shown efficacy in cases with severe organ involvement 4

Risk Factors for Relapse and Complications

• Predictive factors for relapse include:

  • Older age at onset
  • Persistent rash
  • Abdominal pain
  • Hematuria
  • Underlying disease at onset 3
  • Histologically: severe leukocytoclasis and absence of IgM deposit 3

• Patients with severe GI involvement have higher risk of renal involvement and biopsy-proven nephritis 1

Special Considerations

• Pregnancy: Women with history of childhood HSP require close monitoring during pregnancy for proteinuria and hypertension 2

• Long-term follow-up: Required for at least 6 months, with regular urinalysis and blood pressure checks 2

Treatment Algorithm

1. Mild HSP (skin rash only or with mild joint pain)

   • Supportive care and symptomatic treatment
   • Regular monitoring for complications

2. Moderate HSP (significant joint pain, mild GI symptoms)

   • NSAIDs for joint pain
   • Consider short course of oral corticosteroids if symptoms are distressing
   • Close monitoring for renal involvement

3. Severe HSP (significant GI symptoms, renal involvement)

   • Hospitalization may be required
   • Systemic corticosteroids
   • Consider immunosuppressive therapy for organ involvement
   • Specialist consultation (nephrology, gastroenterology)

4. Relapsing HSP

   • Consider colchicine
   • Evaluate for underlying triggers
   • More aggressive immunosuppression may be needed for severe cases

Remember that while the rash is the most visible manifestation of HSP, management should focus on monitoring and treating potential systemic complications, particularly renal involvement, which is most likely to result in long-term morbidity.