Acute Haemorrhagic Oedema of Infancy

Question 1

What age group is primarily affected by acute hemorrhagic oedema of infancy?

Answer 1

Children 4-24 months

Most cases occur in this age range.

Question 2

What are the characteristic skin lesions of acute hemorrhagic oedema of infancy?

Answer 2

Annular, purpuric and targetoid plaques on face/extremities

Lesions can also occur on the trunk.

Question 3

What type of edema is associated with acute hemorrhagic oedema of infancy?

Answer 3

Tender, non-pitting oedema of acral sites

Question 4

Is extrcutaneous involvement common in acute hemorrhagic oedema of infancy?

Answer 4

Rare

Question 5

What is the typical course of acute hemorrhagic oedema of infancy?

Answer 5

Benign course with spontaneous resolution within 1-3 weeks

Question 6

What percentage of cases of acute hemorrhagic oedema of infancy are in boys?

Answer 6

70%

Question 7

What is the aetiology of acute hemorrhagic oedema of infancy?

Answer 7

Unknown, but 75% have associated infection/drug/immunisation

Question 8

What percentage of children with acute hemorrhagic oedema of infancy typically do not appear ill?

Answer 8

Typically do not appear ill, but 45% have fever

Question 9

How long is the typical duration between the initiating event and the onset of the disease?

Answer 9

1-2 weeks

Question 10

Describe the lesions of acute hemorrhagic oedema of infancy take?

Answer 10

• Lesions = Annular, purpuric and targetoid plaques on face/extremities
  ○ Can occur on trunk
  ○ Usually asymptomatic, but can be tender or itchy
  ○ Can be arcuate, polycyclic, rosette-shaped, scalloped, vesicobullous and heal with atrophic scarring
  ○ Evolve into medallion, annular, iris or targetoid plupuric plaques

Lesions can heal with atrophic scarring.

Question 11

What is a rare mucosal involvement that may occur in acute hemorrhagic oedema of infancy?

Answer 11

Oral petechiae, conjunctival injection, abdominal pain, arthritic symptoms, glomerulonephritis, intussusception

Question 12

What is the pathology associated with acute hemorrhagic oedema of infancy?

Answer 12

LCCV with IgA deposition on DIF at capillaries and postcapillary venules in 1/4 - 1/3 of cases

Question 13

What are some differential diagnoses for acute hemorrhagic oedema of infancy?

Answer 13

• HSP and other CSVV
• Other vasculitides
• EM
• Urticaria multiforme
• Urticarial vasculitis
• Kawasaki’s
• Purpura fulminans
• Meningococcal
• Sweets
• Trauma

Question 14

What is the treatment approach for acute hemorrhagic oedema of infancy?

Answer 14

Symptomatic management

May need antibiotics if there is a concurrent infection.

Question 15

Are steroids shown to be helpful in treating acute hemorrhagic oedema of infancy?

Answer 15

No