Medical Care
No effective therapy exists for acute hemorrhagic edema of infancy (AHEI). The use of steroids and antihistamines has been controversial, and they do not appear to alter the disease course. However, systemic corticosteroids may be used to ameliorate the acute manifestations of the disease. [31] Treatment is symptomatic; discontinue antibiotics after obtaining negative culture results.
Inpatient care is not usually required unless the diagnosis of acute hemorrhagic edema of infancy (AHEI) is in doubt. If meningococcemia or another significant condition remains in the differential diagnosis, patients may require monitoring or therapy as appropriate for those disorders.
Consultations
Consult a dermatologist if the diagnosis of acute hemorrhagic edema of infancy (AHEI) is in doubt. Additionally, consult a gastroenterologist or nephrologist if significant abdominal symptoms or renal involvement is noted.
Diet
Acute hemorrhagic edema of infancy (AHEI) patients usually are nontoxic in appearance. Although visceral involvement is rare, maintain a relatively bland diet with plenty of fluids to maintain hydration.
Activity
No particular restrictions in activity are required for acute hemorrhagic edema of infancy (AHEI).
Prevention
No known method exists for preventing acute hemorrhagic edema of infancy (AHEI) or recurrences of the condition.
Long-Term Monitoring
Treatment for acute hemorrhagic edema of infancy (AHEI) is symptomatic. Monitor patients for abdominal or renal involvement, which, although rare, has been reported.
-
Large cockade (rosette or knot of ribbons), annular, or targetoid purpuric lesions found primarily on the face, ears, and extremities are characteristic of acute hemorrhagic edema of infancy.
-
The left leg in this patient with acute hemorrhagic edema of infancy is markedly more edematous than the right leg.
-
Leukocytoclastic vasculitis and fibrinoid necrosis is seen in patients with acute hemorrhagic edema of infancy. This histologic pattern also is seen in Henoch-Schönlein purpura, although patients with Henoch-Schönlein purpura usually have immunoglobulin A deposition, and immunoglobulin A deposition is demonstrable in only approximately one third of patients with acute hemorrhagic edema of infancy (hematoxylin and eosin, magnification X40).
-
This toddler with acute hemorrhagic edema of infancy has a discoloration in the area of the umbilicus similar to that described as Cullen sign.
-
Note the concentric arcs of purpura on the patient's arm.
-
Despite the frightening appearance of purpura in these patients, they usually are in no significant distress.
