Armando Hasudungan
Biology and Medicine videos

Henoch-Schönlein purpura (Spring Fever)

Overview

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.

Signs and symptoms

Henoch Schonlein Purpura Tetrad Palpable purpuric petechial rash, abdominal pain, kidney involvement and arthralgia
  • Recent mucosal infection (respiratory or gastrointestinal)
  • Rash
  • Abdominal pain
  • Nephritis
  • Arthrlagia of large joints
  • Oedema of extremities
  • Hypertension (due to kidney involvement)
Side note Kidney involvement in HSP is similar to IgA nephropathy

Differential 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

Management

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

Summary