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Henoch-Schonlein Purpura

Henoch-Schonlein Purpura is also known as IgA vasculitis, and it presents with a purpuric rash on the lower limbs and buttocks in children. IgA deposits in blood vessels, causing inflammation in the affected organs e.g. skin, kidneys, gut.


Presentation

  • Often preceded by an URTI or Gastroenteritis

  • Purpura (100%) – In lower limbs and buttocks

  • Arthralgia (75%) – In knees and ankles

  • Abdominal pain (50%) – Severe cases can lead to haemorrhage, intussusception or bowel infarction

  • IgA Nephritis (50%) – Haematuria +/- Proteinuria

    • If 2+ protein on urine dip, the patient has developed nephrotic syndrome, and will have some degree of oedema

“Henoch–Schönlein purpura” © Madhero88 CC BY-SA 3.0 (https://creativecommons.org/licenses/by-sa/3.0/)

Investigations

  • Exclude other serious pathologies, like meningococcal septicaemia and leukaemia

  • Bloods – FBC, CRP, U&E, Albumin

  • Blood film and culture

  • Urine dip

  • BP – for HTN due to renal involvement


Diagnostic Criteria

The patient has to have Purpura + at least 1 of:

  • Arthralgia or Arthritis

  • Diffuse abdominal pain

  • Haematuria or Proteinuria

  • IgA deposits on histology


Management

  • Supportive with simple analgesia, rest and hydration

  • NSAIDs for analgesic and anti-inflammatory effects

  • Close monitoring during active illness with Urine dips and BP to check for renal impairment


Prognosis

For most patients, the condition is self-limiting, with most recovering completely, but 1/3 having a recurrence of symptoms. The long-term prognosis is mainly related to the extent of renal involvement as. Those with nephrotic syndrome, renal impairment or secondary HTN at presentation are likely to have a poorer outcome.



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