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Key Features

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Essentials of Diagnosis
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  • Purpuric cutaneous rash

  • Migratory polyarthritis or polyarthralgias

  • Intermittent abdominal pain

  • Nephritis

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General Considerations
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  • Most common type of small vessel vasculitis in children

  • Primarily affects boys 2–7 years of age

  • Occurrence is highest in the spring and fall

  • Upper respiratory infection precedes the diagnosis in two-thirds of children

  • Antigens from group A β-hemolytic streptococci and other bacteria, viruses, drugs, foods, and insect bites have been proposed as inciting agents

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Clinical Findings

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Symptoms and Signs
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  • Skin involvement

    • May be urticarial initially

    • Progresses to maculopapules

    • Coalesces to a symmetric, palpable purpuric rash distributed on the legs, buttocks, and elbows

  • New lesions may continue to appear for 2–4 weeks, and may extend to involve the entire body

  • Migratory polyarthralgias or polyarthritis, primarily of the ankles and knees develop in two-thirds of patients

  • Intermittent, sharp abdominal pain occurs in approximately 50% of patients, and hemorrhage and edema of the small intestine can often be demonstrated

  • Intussusception may develop

  • Renal involvement

    • Develops during second or third week of illness with either a nephritic or, less commonly, nephrotic picture in 25–50% of patients

    • May be accompanied by hypertension

  • In males, testicular torsion may also occur

  • Neurologic symptoms are possible due to small vessel vasculitis

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Differential Diagnosis
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  • The rash of septicemia (especially meningococcemia) may be similar to skin involvement in Henoch-Schönlein purpura, although the distribution tends to be more generalized

  • The possibility of trauma should be considered in any child presenting with purpura

  • Other vasculitides should also be considered

  • The lesions of thrombotic thrombocytopenic purpura are not palpable

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Diagnosis

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  • Platelet count is normal or elevated

  • Other screening tests of hemostasis and platelet function are typically normal

  • Urinalysis frequently reveals hematuria and sometimes proteinuria

  • Stool may be positive for occult blood

  • The antistreptolysin O (ASO) titer is often elevated and the throat culture positive for group A β-hemolytic streptococci

  • Serum IgA may be elevated

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Treatment

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  • Supportive

  • Nonsteroidal anti-inflammatory drugs may be useful for the arthritis

  • Corticosteroid therapy may provide symptomatic relief for severe gastrointestinal or joint manifestations but does not alter skin or renal manifestations

  • If culture for group A β-hemolytic streptococci is positive or if the ASO titer is elevated, a therapeutic course of penicillin is warranted

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Outcome

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Prognosis
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  • Generally good, although symptoms frequently (25–50%) recur over a period of several months

  • Microscopic hematuria may persist for years in patients with renal manifestations

  • Progressive kidney failure occurs in < 5%, with an overall fatality rate of 3%

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Reference

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McCarthy  HJ: Clinical practice: diagnosis and management of Henoch-Schönlein purpura. Eur J Pediatr 2010;169:643–650
[PubMed: 20012647]

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