Henoch-Schönlein Purpura

For further information, see CMDT Part 20-25: Henoch-Schönlein Purpura

Key Features

• The most common systemic vasculitis in children

• Occurs in adults as well

Clinical Findings

• Purpuric skin lesions typically located on the lower extremities; may also be seen on the hands, arms, trunk, and buttocks

• Joint symptoms are present in most patients; the knees and ankles are most commonly involved

• Abdominal pain secondary to vasculitis of the intestinal tract is often associated with gastrointestinal bleeding

• Hematuria signals the presence of a glomerular lesion that is usually reversible, although it occasionally may progress to chronic kidney disease

Diagnosis

• Skin biopsy can demonstrate leukocytoclastic vasculitis with IgA deposition

• Kidney biopsy reveals segmental glomerulonephritis with crescents and mesangial deposition of IgA

• Differential diagnosis

  • Immune thrombocytopenia

  • Meningococcemia

  • Rocky Mountain spotted fever

  • Rheumatoid arthritis (including juvenile form)

  • Polyarteritis nodosa

  • Endocarditis

  • Cryoglobulinemia

Treatment

• Chronic courses with persistent or intermittent skin disease are more likely to occur in adults than children

• The efficacy of treatment is not well established

• In children, prednisone (1–2 mg/kg/day orally) does not decrease the frequency of proteinuria 1 year after onset of disease

• Severe disease is often treated with aggressive immunosuppressive agents such as mycophenolate mofetil, but there is no consensus as to the efficacy of this approach or the optimal therapeutic regimen