Acute Hemorrhagic

 Edema of Infancy

 AHEI is a distinctive, cutaneous, small vessel leukocytoclastic vasculitis of young children with dramatic characteristic skin findings. The cutaneous findings are dramatic both in appearance and rapidity of onset. The 2 primary features include large cockade (rosette or knot of ribbons), annular, or targetoid purpuric lesions found primarily on the face, ears, and extremities, and edema of the limbs and face

The typical patient with AHEI is aged 4-24 months with a history of recent upper respiratory tract illness and/or course of antibiotics. Associated fever is common but tends to be low grade, and despite the impressive clinical presentation, patients usually are nontoxic in appearance. Visceral involvement is uncommon, and spontaneous recovery usually occurs within 1-3 weeks, without sequelae. Recurrent episodes may occur

Target lesions in AHEI usually are limited to limbs and the face, and progressive extremity edema occurs in AHEI that does not occur in patients with erythema multiforme 

.The petechiae and purpura also may resemble lesions of HSP and meningococcemia, although patients often appear much sicker with high fever, malaise, and possible shock in severe cases of meningococcemia. Most patients improve with no residual skin lesions; however, AlSufyani reported a patient with AHEI who resolved with unusual scarring

 Angioedema may be found in patients with HSP, but usually not in erythema multiforme or meningococcemia

 The duration of illness varies in patients with meningococcemia, but patients with AHEI usually are back to baseline within 2-3 weeks; patients with HSP and erythema multiforme usually are better by 4-6 weeks.

The treatment for AHEI and erythema multiforme is supportive; prednisone is controversial for HSP, and intravenous penicillin is the drug of choice for meningococcemia.

Pathophysiology

Acute hemorrhagic edema of infancy (AHEI) is a distinct variety of leukocytoclastic vasculitis. Leukocytoclastic vasculitis is probably mediated by immune complexes. Deposition of immunoglobulin A (IgA) is common in patients with Henoch-Schönlein purpura (HSP) but is observed in less than one third of skin biopsy specimens from patients with AHEI

Clinical

History

• Age of onset for acute hemorrhagic edema of infancy (AHEI) usually is 4-24 months.
• Clinical findings develop rapidly over 24-48 hours.
• Respiratory tract infection, drug intake, or vaccination frequently precedes AHEI.
• Fever is common but tends to be low grade.
• Patients usually are reported to have been in minimal distress despite the dramatic appearance of skin findings.
• Reports of joint or abdominal pain are uncommon.
• Recurrent episodes may occur.
• Edema develops early in the course of the disorder and frequently involves the dorsum of the hands and feet, extending proximally up the extremities. Edema of the scalp has been reported.

Physical

The clinical picture for acute hemorrhagic edema of infancy (AHEI) is quite typical.

• Patients usually are nontoxic in appearance.
• Characteristic, large, cockade, annular, or targetoid purpuric lesions are found primarily on the face, ears, and extremities.
  • Purpura may involve the scrotum.
  • Lesions may begin as urticarial plaques and enlarge up to 5 cm in diameter. The borders are sharp.
  • Mucosal involvement is rare but has been reported.
• Purpura of the umbilicus can be mistaken for Cullen sign

• Acral edema involving the dorsum of the hands and feet frequently extends proximally up the extremities.
• Edema is nontender and may be asymmetric.
• Associated fever tends to be low grade.
• Joint and abdominal examinations are unremarkable.
• Involvement of internal organs is uncommon.

Causes

• The cause of acute hemorrhagic edema of infancy (AHEI) is unknown; however, it is most frequently preceded by respiratory tract infections. In addition, numerous other infections, drug intake, and vaccinations have been implicated.^24,25,26,27
• AHEI probably is an immune complex disorder; however, immune complexes have been demonstrated in only some cases.^18,28

Most cases of AHEI occur during the winter months

Treatment

Medical Care

No effective therapy exists for acute hemorrhagic edema of infancy (AHEI). Systemic steroids do not appear to alter disease course. Treatment is symptomatic; discontinue antibiotics after obtaining negative culture results.

Consultations

• Consult a dermatologist if the diagnosis of acute hemorrhagic edema of infancy (AHEI) is in doubt.
• 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).