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Henoch-Schönlein purpura (HSP) is an IgA-mediated, autoimmune hypersensitivity vasculitis of childhood. The main clinical features are skin purpura, arthritis, abdominal pain, gastrointestinal bleeding, orchitis and nephritis. The aetiology remains unknown.
The male-to-female ratio is 1.5-2:1. Caucasians are more often affected than other ethnic groups.
Associated conditions preceding HSP include:
- Infections: for example, Group A streptococci, mycoplasma, Epstein-Barr virus
- Environmental exposures: for example, drug and food allergens, pesticides, cold exposure, insect bites
- The disease occurs mostly in the winter months. About 50-90% of patients have a preceding upper respiratory tract infection (URTI).
- Generally, patients appear to be mildly ill, with low-grade fever.
- There is a symmetrical, erythematous macular rash, especially on the back of the legs, buttocks and ulnar side of the arms.
- Within 24 hours, the macules evolve into purpuric lesions, which may coalesce and resemble bruises.
- Abdominal pain and bloody diarrhoea may precede the typical purpuric rash. Intussusception should then be considered (occurs in 2-3% of patients). HSP may also cause nausea and vomiting.
- Joint pain, especially in the knees and ankles. Joints may also be swollen and tender but permanent deformity does not occur.
- Renal involvement:
- Affects 50% of older children and 25% of children under 2 years of age
- Less than 1% of patients progress to end-stage renal disease
- Usually occurs within three months of disease onset
- There is usually no relationship between the severity of nephritis and the extent of the other manifestations of HSP
- Microscopic haematuria with mild-to-moderate proteinuria may occur
- Nephrotic syndrome may also occur
- Oliguria and hypertension are rare
- Scrotal involvement may mimic testicular torsion.
- Neurological: headaches may occur.
- Connective tissue diseases - eg, systemic lupus erythematosus (SLE)
- Other causes of purpuric rash - eg, thrombocytopenia
- Other causes of glomerulonephritis
- Acute haemorrhagic oedema of infancy is milder and occurs in infants younger than 2 years. The onset is sudden, with acute purpura, bruises and tender oedema of the limbs and face
Diagnosis of HSP is clinical and not based on laboratory investigations. The following abnormalities may be present:
- Urinalysis: haematuria and/or proteinuria are present in 20-40% of patients
- FBC: there may be raised white cell count with eosinophilia; normal or increased platelets
- Raised ESR
- Serum creatinine may be elevated in renal involvement
- Serum IgA levels are often increased
- Autoantibody screen: connective tissue diseases
- Abdominal ultrasound: if there are gastrointestinal symptoms - for diagnosis of intestinal obstruction
- Barium enema: may be used to confirm and treat intussusception
- Testicular ultrasound: assessment of possible torsion
- Renal biopsy: if there is persistent nephrotic syndrome
- HSP is usually self-limiting and no form of therapy has been shown appreciably to shorten the duration of disease or prevent complications. Therefore, treatment for most patients remains primarily supportive.
- Non-steroidal anti-inflammatory drugs (NSAIDs) may help joint pain but should be used with caution in patients with renal insufficiency.
- May require admission to hospital for monitoring of abdominal and renal complications.
- Nephropathy: treated supportively. A variety of drugs (steroids, azathioprine, cyclophosphamide) and plasmapheresis have been used to prevent the progression of the renal disease but the results of trials have been inconsistent.
- Corticosteroids can ameliorate associated arthralgias and the symptoms associated with gastrointestinal dysfunction. There was no evidence of benefit of prednisone in preventing serious long-term kidney disease in HSP. 
- No controlled clinical trials have been performed with immunosuppressive drugs, although azathioprine or cyclophosphamide may be beneficial.
- Plasma exchange is used in the management of some adults with vasculitis and idiopathic rapidly progressive nephritis.
- Renal involvement occurs in 50% of older children but is only serious in approximately 10% of patients. Less than 1% of patients with HSP progress to end-stage renal failure. The renal prognosis is worse in older children and adults.
- Monitoring for renal involvement:
- For those with no proteinuria, recommendations are for blood pressure checking and urinalysis at days 7 and 14 and at 1, 3, 6 and 12 months.
- For those with proteinuria, follow-up is recomended at days 7 and 14, monthly from 1-6 months and then at 12 months.
- Other rare complications include myocardial infarction, pulmonary haemorrhage, pleural effusion, intussusception (in 2-3% of patients), gastrointestinal bleeding, bowel infarction, seizures and mononeuropathies.
- Recurrence of symptoms may occur. Recurrence of renal impairment may also occur but is rare.
- HSP is an acute self-limited illness and usually resolves without treatment, but may rarely lead to complications. Initial attacks of HSP can last for several months. One third of patients have one or more recurrences.
- Children younger than 3 years have a shorter, milder course and fewer recurrences.
- The long-term prognosis of HSP is directly dependent on the severity of renal involvement.
Further reading & references
- Henoch-Schönlein purpura; DermNet NZ
- Watson L, Richardson AR, Holt RC, et al; Henoch schonlein purpura--a 5-year review and proposed pathway. PLoS One. 2012;7(1):e29512. doi: 10.1371/journal.pone.0029512. Epub 2012 Jan 3.
- Gardner-Medwin JM, Dolezalova P, Cummins C, et al; Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002 Oct 19;360(9341):1197-202.
- Lane SE, Watts R, Scott DG; Epidemiology of systemic vasculitis. Curr Rheumatol Rep. 2005 Aug;7(4):270-5.
- González, L. M., Janniger, C. K. and Schwartz, R. A. (2009); Pediatric Henoch–Schönlein purpura. International Journal of Dermatology, 48: 1157–1165. doi: 10.1111/j.1365-4632.2009.04162.x
- Shetty AK, Desselle BC, Ey JL, et al; Infantile Henoch-Schonlein purpura. Arch Fam Med. 2000 Jun;9(6):553-6.
- Tizard EJ; Henoch-Schonlein purpura. Arch Dis Child. 1999 Apr;80(4):380-3.
- Weiss PF, Klink AJ, Localio R, et al; Corticosteroids may improve clinical outcomes during hospitalization for Pediatrics. 2010 Oct;126(4):674-81. Epub 2010 Sep 20.
- Chartapisak W, Opastirakul S, Hodson EM, et al; Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database Syst Rev. 2009 Jul 8;(3):CD005128.
- Saulsbury FT; Henoch-Schonlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999 Nov;78(6):395-409.
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|Original Author: Dr Colin Tidy||Current Version: Dr Dave Wilson||Peer Reviewer: Dr Helen Huins|
|Last Checked: 02/05/2013||Document ID: 2251 Version: 22||© EMIS|