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Henoch-Schönlein Purpura (Skin/IgA Vasculitis)

IgA-mediated small-vessel vasculitis: palpable purpura and joint-renal involvement

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Henoch-Schönlein Purpura (Skin/IgA Vasculitis)?

IgA vasculitis is a small-vessel vasculitis with annual incidence of 10-20/100,000 in children (peak 4-7 years); 90% of cases are pediatric. Adult onset is rarer but tends toward more severe renal involvement. Most often follows upper respiratory tract infection (Group A streptococcus, EBV, mycoplasma).

Pathophysiology: galactose-deficient IgA1 (Gd-IgA1) immune complex deposits in vessel walls activate complement and neutrophil-mediated vasculitis. EULAR/PRINTO/PRES criteria require palpable purpura + 1 (abdominal pain, arthralgia/arthritis, renal involvement, or biopsy IgA deposition).

Skin findings: symmetric palpable purpura on lower extremities, buttocks, sometimes on extensor surfaces of arms; non-blanching, may form bullae or ulcers; sometimes urticarial early. Histology: leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. Most disease (>90%) is self-limited, resolving in 4-6 weeks, but renal involvement (30-50%) requires long-term follow-up.

Symptoms

Palpable purpura (lower extremities, gluteal region - bilateral)
Arthralgia/arthritis (knee, ankle - 75%)
Acute abdominal pain (60-65%)
Hematuria/proteinuria (20-50% renal)
Scrotal swelling (in boys)
Headache and seizures (rarely - CNS involvement)

Risk Factors

Childhood (4-7 years peak)
Male gender (1.5-2:1 predominance)
Recent upper respiratory tract infection (GAS, EBV, parvovirus B19)
Vaccination history (especially MMR, influenza)
Drug exposure (antibiotics, NSAIDs)
Cold seasons (autumn-winter)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Symmetric palpable purpura on lower extremities and buttocks
  • Severe abdominal pain (intussusception suspicion)
  • Hematuria, proteinuria, edema
  • Joint pain and limited mobility
  • Scrotal swelling and tenderness
  • Hypertension (renal involvement indicator)

Treatment Methods

01
Supportive (rest, hydration, analgesia) - first line in mild cases
02
Oral prednisolone (1 mg/kg/day) - severe abdominal pain/orchitis/arthritis
03
Pulse methylprednisolone (severe nephritis)
04
Cyclophosphamide, mycophenolate (rapidly progressive nephritis)
05
ACE inhibitor/ARB (proteinuria management)
06
Long-term renal follow-up (urinalysis, BP, renal function for 5 years)

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.