Who should I consult for Henoch-Schönlein Purpura (HSP) rash?

Management of Henoch-Schönlein Purpura (HSP) Rash

For Henoch-Schönlein Purpura (HSP) rash, patients should be referred to a rheumatologist for primary management, with dermatology consultation for skin manifestations and nephrology referral if renal involvement is present.

Understanding HSP

HSP (also called IgA vasculitis) is a systemic small-vessel vasculitis characterized by:

• Palpable purpura predominantly on the lower extremities
• Potential involvement of multiple organ systems including:
  • Gastrointestinal tract (abdominal pain, bleeding)
  • Joints (arthritis/arthralgia)
  • Kidneys (hematuria, proteinuria)

Appropriate Specialist Referral

Primary Management:

• Rheumatologist: Should lead the multidisciplinary care team as HSP is fundamentally a systemic vasculitis 1
  • Rheumatologists are trained in the diagnosis and management of vasculitic conditions
  • They can coordinate care between different specialists

Additional Specialist Referrals:

• Dermatologist: For evaluation and management of severe or atypical skin manifestations 2

  • Can perform skin biopsy to confirm diagnosis when needed
  • Can help manage persistent or severe cutaneous symptoms

• Nephrologist: Essential if there are signs of renal involvement 1, 3

  • Indicated when patient has hematuria or proteinuria
  • Renal involvement is the most important prognostic factor determining long-term morbidity and mortality

Diagnostic Approach

The diagnosis of HSP is based on:

1. Palpable purpura (mandatory criterion)
2. Plus at least one of the following:
   • Diffuse abdominal pain
   • Arthritis or arthralgia
   • Renal involvement (hematuria and/or proteinuria)
   • Biopsy showing predominant IgA deposition 1

Treatment Considerations

Treatment depends on organ involvement and severity:

• Most cases are self-limited with an average duration of 4 weeks 1
• General supportive care is the mainstay of treatment for mild cases
• For severe gastrointestinal involvement (pain, bleeding):
  • Oral corticosteroids may be considered 4
• For severe nephritis or persistent proteinuria >3 months:
  • Consider ACE inhibitors or ARBs in addition to corticosteroids 1
  • In rare severe cases, immunosuppressants may be needed 5

Follow-up Recommendations

• All patients should be monitored for at least 6 months after diagnosis
• Regular urine testing for proteinuria and hematuria
• Blood pressure monitoring
• Particular attention to women with history of HSP during pregnancy due to increased risk of complications 3

Common Pitfalls to Avoid

1. Failing to monitor for renal involvement, which is the most significant determinant of long-term prognosis
2. Assuming HSP is always benign and self-limited without appropriate follow-up
3. Overlooking potential triggers (infections, medications) that should be addressed
4. Treating all cases with corticosteroids, which is not supported by evidence for preventing nephropathy 1

By ensuring appropriate specialist referral and systematic monitoring, patients with HSP can receive optimal care that addresses both the acute manifestations and potential long-term complications of this vasculitis.