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
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.
