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Henoch-Schonlein Purpura

  • Introduction

    HSP typically presents with the triad of 

    • purpuric rash on the extensor surfaces of limbs (mainly lower) and buttocks, 
    • joint pain/swelling and 
    • abdominal pain. 

    Abdominal pain or arthralgia sometimes precede the rash.The commonest age group is 2 - 8 years. The cause is unknown but there may be a recent history of an upper respiratory tract infection.

    Assessment

    • Purpura: If atypical distribution or the child is unwell, consider meningococcaemia, thrombocytopenia, or other rare vasculitides.
    • Joint Pain: Swelling and arthralgia of large joints are often the patient's main complaint. In most situations this pain resolves spontaneously within 24-48 hours.
    • Abdominal pain: Uncomplicated abdominal pain often resolves spontaneously within 72 hours. However serious abdominal complications may occur including intussusception, bloody stools, haematemesis, spontaneous bowel perforation, and pancreatitis.
    • Renal disease: Haematuria is present in 90% of cases, but only 5% are persistent or recurrent. Less common renal manifestations include proteinuria, nephrotic syndrome, isolated hypertension, renal insufficiency and renal failure (<1%). Renal involvement may only present during the convalescent period.
    • Subcutaneous oedema (scrotum, hands, feet, sacrum): This can be very painful.
    • Rare complications - pulmonary and CNS involvement

    Typical rash distribution

    Typical rash distribution

    Investigations

    Urine analysis should be performed, and if haematuria is present the sample should be sent for microscopy to quantify the RBC count.

    Other investigations may include:

    • Full blood count
    • Urea, electrolytes with creatinine
    • Blood culture

    Management

    All patients presenting with a purpuric rash must be seen by a consultant or registrar, even if the child does not appear unwell.

    • Document the child's blood pressure
    • Consider a surgical consult if abdominal features are prominent. Testicular torsion can be hard to differentiate from the pain of vasculitic testicular pain.
    • The use of prednisolone has not been shown to make clinically important improvements in the rate of long-term renal complications.  It has been shown to reduce the duration of abdominal and joint pain and may reduce the risk of abdominal complications. It may be considered for use in patients with more than mild joint or abdominal pain. Consider prednisolone 1mg/kg while symptoms persist.

    Indications for admission:

    abdominal complication. Arrange early surgical consultation.

    renal complication eg nephritis, nephrotic syndrome

    Also consider admission for symptomatic treatment:

    • severe joint pain - treatment is bed rest and analgesia
    • abdominal pain
    • painful subcutaneous oedema

    Disposition

    Give the family a Parent Information Sheet on HSP

    If discharged from the ED then it is imperative that appropriate follow-up is arranged to ensure adequate symptom control and resolution of the disease. Short term support can occur in the ED but follow-up care should soon be transferred to the GP (emphasise the need for ongoing BP and urine review in the letter) or a paediatrician - an appointment may be made in the General Medical Outpatient Clinic. The rash is usually the last manifestation to remit and appears to worsen if the child is very active. Some recommend an annual BP and urinalysis for life.

    As the renal involvement can present up to six months after the initial presentation the urine should be checked regularly for that period. The blood pressure should be checked twice during that time. If the child has persistent renal involvement they should be referred to a paediatrician, or paediatric nephrologist for long term follow up.

    Parent Information Sheet  (Print version - PDF)

    Parent Information Sheet  (HTML version)

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