Acute hemorrhagic edema of infancy
Although the cause is unknown, it is thought to be an immune complex-related reaction to infection, drugs, or vaccination. Some consider it to be a variant of immunoglobulin A vasculitis (IgAV, formerly Henoch-Schönlein purpura); there are cases of children aged 2-4 years who have symptoms overlapping AHEI and IgAV.
AHEI is uncommon but reported worldwide. Most cases occur in winter.
Edema of the face is often seen initially, suddenly followed by skin lesions beginning distally and spreading proximally. The patient may have a low-grade fever but does not appear toxic. Systemic symptoms are rare, although joint, gastrointestinal (with bloody diarrhea), and renal (with hematuria and proteinuria) involvement may occur. Purpuric lesions can be painful.
Illness lasts 1-3 weeks and resolves without consequence. Recurrences may occur.
D69.0 – Allergic purpura
P83.39 – Other edema specific to newborn
402856005 – Acute hemorrhagic edema of childhood
Differential Diagnosis & Pitfalls
- IgAV – Typically presents with more systemic involvement (with abdominal pain, arthritis, and nephritis).
- Erythema multiforme minor – Target lesions in AHEI are usually limited to the limbs and face, and progressive extremity edema that occurs in AHEI does not occur in patients with erythema multiforme.
- Acute meningococcemia – Patients often appear much sicker, with high fever, malaise, and possible shock.
- Kawasaki disease
- Child abuse
- Autoinflammatory disease – See, eg, TNF receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndromes, and CANDLE syndrome, among others.