eMedicine Specialties > Dermatology > Pediatric Diseases
Acute Hemorrhagic Edema of Infancy
Updated: Sep 25, 2009
Introduction
Background
Snow1 first described acute hemorrhagic edema of infancy (AHEI) in the United States in 1913. Del Carril, Diaz Sobillo, and Vidal2 described the condition in Argentina in 1936. Europeans have recognized Finkelstein's description of this disease since his publication in 1938,3 and, until recently, most reports of this disorder occurred in the European literature under the terms Finkelstein disease, Seidlmayer syndrome,4 or purpura en cocarde avec oedema.
AHEI is a distinctive, cutaneous, small vessel leukocytoclastic vasculitis of young children with dramatic characteristic skin findings.5,6,7,8 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 (see Media File 1 ).
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 typical patient with AHEI is aged 4-24 months with a history of recent upper respiratory tract illness and/or course of antibiotics.9 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.
Many physicians believe AHEI is a mild variant of Henoch-Schönlein purpura (HSP); others believe it to be a unique disorder.10,11,12 Gattorno et al13 reported a brother and sister aged 3.6 years and 16 months, respectively. Both had pharyngitis treated with amoxicillin for 10 days, which occurred 20 days before admission. The 3.6-year-old girl developed a purpuric rash on her legs and abdominal pain, and HSP was diagnosed.
Subsequently, 3 days later, her brother developed a widespread rash consistent with the diagnosis of AHEI. Neither joint effusions nor abdominal symptoms were present. Cases also exist of children aged 2-4 years who appear to have symptoms with overlap between AHEI and HSP.
Regardless, AHEI may have a clinical presentation distinct from HSP seen in older children. The condition has a much better prognosis in these children.
HSP usually presents with palpable purpura or petechiae associated with 1 or more symptoms, including abdominal pain, arthritis/arthralgias, and nephritis; however, any of these symptoms may be absent, which often leads to confusion in diagnosing the condition. The diagnosis may be particularly difficult to make when a patient presents with isolated symptoms, such as abdominal pain without the typical rash. Scalp edema and/or scrotal swelling also may be seen in patients with HSP.
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.14
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.
Frequency
United States
Acute hemorrhagic edema of infancy (AHEI) is uncommon in the United States. Specific frequency data have not been reported.
International
Until recently, most reports of acute hemorrhagic edema of infancy (AHEI) occurred in the European literature under the terms Finkelstein disease, Seidlmayer syndrome, or purpura en cocarde avec oedema. The disorder is uncommon but has been reported in countries throughout the world.
Mortality/Morbidity
Acute hemorrhagic edema of infancy (AHEI) usually is benign and without sequelae, with spontaneous recovery occurring within 1-3 weeks. Arthritis, nephritis,15,16 abdominal pain, gastrointestinal tract bleeding, and lethal intestinal complications rarely are reported.17 Recurrences may occur. AlSufyani reported a patient with AHEI who resolved with unusual scarring.14
Race
No racial predilection has been described for acute hemorrhagic edema of infancy (AHEI).
Sex
Acute hemorrhagic edema of infancy (AHEI) is slightly more common among male infants than among female infants.18
Age
Age of onset for acute hemorrhagic edema of infancy (AHEI) usually is 2-60 months (median, 11 mo).18,19
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.20,21
- Purpura of the umbilicus can be mistaken for Cullen sign (see Media File 4).
This toddler with acute hemorrhagic edema of infancy has a discoloration in the area of the umbilicus similar to that described as 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.
The left leg in this patient with acute hemorrhagic edema of infancy is markedly more edematous than the right leg.
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.
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.
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| References |
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References
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Further Reading
Keywords
acute hemorrhagic edema of infancy, AHEI, acute infantile hemorrhagic oedema, Finkelstein's disease, Seidlmayer syndrome, , cockade purpura with edema, postinfectious cockade purpura of early childhood, acute benign cutaneous leukocytoclastic vasculitis of infancy
