Overview

Henoch Schonlein Purpura is a IgA vasculitis characterised by the Tetrad: palpable purpuric petechial rash, abdominal pain, kidney involvement and arthralgia. Aetiology is unknown but thought to have a genetic predisposition.

Clinical Manifestation

• Recent mucosal infection (respiratory or gastrointestinal)
• Rash
• Abdominal pain
• Nephritis
• Arthrlagia of large joints
• Oedema of extremities
• Hypertension (due to kidney involvement)

Diagnosis

Differential Diagnosis of Purpura/Petechiae in a child

• Thrombocytopaenia
  • Decreased platelet production
    • Infections
    • Drugs and alcohol
    • Aplastic anaemia
    • Leukaemia
    • Myelodysplastic syndrome
  • Increased platelet destruction
    • Immune mediated – Immune thrombocytopaenic purpuura
    • Non-immune mediated – causes of microangiopathic haemolysis (TTC, HUS, DIC, pre-eclampsia, HELLP)
  • Dilutional thrombocytopaenia – from massive blood transfusion
  • Distributional thrombocytopaenia – caused by splenic sequestration
    • Portal hypertension
• Non-thrombocytopaenia
  • Vasculitis
    • Henoch Schonlein pupura
    • Wegener’s granulomatosis
  • Hand, foot and mouth disease (Coxsackie virus)
  • Meningococcemia

Investigation

• Urinalysis
  • Haematuria
  • Proteinuria
• Urine culture
• Blood culture
• FBC
• EUC
• Albumin
• Screen for nephritic differentials
  • ANA
  • Anti-DNA
  • Anti-Smiths
• ESR/CRP
• Ultrasound
• PT/APPT – normal because it is not a clotting problem it is a vascular problem

Treatment

Acute management

• Mild pain
  • Paracetamol
  • NSAIDs (if not older)
  • Elevate oedematous lower limb
• Moderate-severe pain
  • Admit
  • IV steroids OR oral steroids
  • Paediatric consult
  • Elevate oedematous lower limb

Ongoing management

• Regular GP/Paediatrician review for progress and complications

Complications and Prognosis

• Bowel infarction
• Bowel perforation
• Intussusception
• PR bleeding
• Respiratory disease
• Nephritic/Nephrotic syndrome
• Intracranial Haemorrhage