Henoch-Schönlein Purpura (HSP) is the most common cause of non-thrombocytopenic purpura in children. The cause is unknown, but it is an IgA-mediated vasculitis of small vessels, usually following an upper respiratory tract infection (URTI).
It is more common in children than in adults, with a peak incidence in the 2-8 year age group, and a seasonal peak in winter.
The onset of the illness may be acute, with simultaneous involvement of several organ groups, or insidious, with sequential occurrence of features over weeks to months.
It is usually a self-limiting disease.
Many children will need only a urine test and can be sent home with GP follow up.
The child is generally well-looking
There is often a history of a recent URTI
Small-vessel vasculitis may involve several organ groups, in particular the skin, joints, gastrointestinal tract and kidneys
Rarely, there may be CNS or pulmonary involvement
Initially blanching pink maculopapules which may be discrete or confluent
Progresses to petechiae or purpura, which are often raised (“palpable purpura”)
The distribution of the purpura typically involves gravity-dependent body areas, particularly the buttocks, legs and extensor surfaces of the arms.
Vasculitis of dermal vessels also results in angio-oedema
Non-pitting, often painful, oedema of dependent areas (hands and feet) as well as the eyelids, lips or scrotum.
Occurs in two-thirds of patients
Self-limiting serous joint effusions which resolve over several days
Typically involving the gravity-dependent joints (elbows, wrists, knees, ankles) – swollen, painful joints; may cause difficulty in weight-bearing
Abdominal pain is common, and is generally intermittent and colicky in nature
Diarrhoea (with occult blood) is common
Occasionally, frank haematemesis or melaena may occur
More serious, but infrequent, complications include spontaneous bowel perforation or intussusception
90% of patients have microscopic haematuria, but this is persistent or recurrent in only 5% of cases
Serious complications may include acute glomerulonephritis, nephrotic syndrome, acute renal failure (<1%), or isolated hypertension
Renal involvement may be a late manifestation, up to 6 months after initial presentation.
Patients initially presenting with only with abdominal pain or arthralgia may pose a diagnostic challenge
Patients initially presenting with haematuria only should have other causes of haematuria excluded
There is no diagnostic investigation for HSP. Investigations are used to detect complications or other causes of purpura
Microscopic haematuria is common (90%)
If macroscopic haematuria is present, urine should be checked for RBC casts (nephritis) and protein (nephrotic syndrome)
FBC to exclude thromocytopaenia as cause of purpura if diagnosis is uncertain
Blood culture, white cell count, CRP, meningococcal PCR if meningococcal septicaemia is suspected
Urea, creatinine and electrolytes if renal impairment is suspected
Complement levels and ASO-titres if nephritis is present
Most patients with HSP will be managed as an outpatient with symptomatic treatment (analgesia) and follow up.
Surgical consult for abdominal complications or testicular pain (testicular pain from vasculitis may be difficult to differentiate from testicular torsion)
Abdominal X-Ray or ultrasound may be needed to exclude abdominal complications
There is some evidence to suggest that prednisolone 1 mg/kg/day for 2 weeks benefits in abdominal and joint pain (and oedema)
There is very limited evidence in preventing renal complications
The decision whether to start prednisolone should be made by the admitting Paediatric Consultant
Abdominal complications (intussusception, perforation, haematemesis or bloody stools)
Renal complications (nephritis, nephrotic syndrome, hypertension, renal failure)
Symptomatic relief (severe joint pain or abdominal pain, painful oedema)
Referrals and follow-up
Patients should receive follow up with their GP or Paediatrician for at least 6 months to ensure symptom relief and disease resolution
Check BP and urinalysis weekly for one month, then monthly for 6 months after presentation as renal disease may present late
If still present at 6 months refer to the Renal team
Henoch-Schonlein Purpura Health Facts
Routine nursing care.
Dudley J, Smith G, Llewelyn-Edwards A, Bayliss K, Pike K and Tizard J. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Archives of Disease in Childhood 2013, July. Accessed online:
http://adc.bmj.com/content/early/2013/07/10/archdischild-2013-303642.abstract Shin JI and Lee JS. Steroids in Henoch-Schonlein purpura and abdominal pain. Archives of Disease in Childhood. 2006 Aug; 91(8):714. Accessed online:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2083041/ Weiss PF, Klink AJ, Localio R, Hall M, Hexam K, Burnham JM, Keren R and Feudtner C. Corticosteroids may improve clinical outcomes during hospitalisation for Henoch-Schonlein purpura. Paediatrics. 2010 Oct, 126 (4): 627-81. Accessed online:
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Author / Reviewer
Kids Health WA Guidelines Team
Dr Meredith Borland HoD, PMH Emergency Department
14 April, 2015
14 April, 2015