Acute hemorrhagic edema of infancy - Skin
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 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, followed suddenly by skin lesions beginning distally and spreading proximally. The patient may have 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.
P83.39 – Other edema specific to newborn
78913002 – Edema of newborn
- Immunoglobulin A vasculitis (IgAV) – Typically presents with more systemic involvement (with abdominal pain, arthritis, and nephritis).
- Erythema multiforme minor – Target lesions in AHEI are usually limited to limbs and the face, and progressive extremity edema occurs in AHEI that does not occur in patients with erythema multiforme.
- Acute meningococcemia – Patients often appear much sicker, with high fever, malaise, and possible shock.
- Urticarial vasculitis – Edema is not as prominent, pruritus may occur, and lesions are not usually as purpuric.
- Kawasaki disease
- Child abuse