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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

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