Henoch-Schönlein purpura (Spring Fever)

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

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)

Kidney involvement in HSP is similar to IgA nephropathy.

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