Acute Hemorrhagic Edema of Infancy

Updated: May 02, 2016
  • Author: Donald Shenenberger, MD, FAAD, FAAFP; Chief Editor: William D James, MD  more...
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Overview

Background

Snow [1] first described acute hemorrhagic edema of infancy (AHEI) as a purely cutaneous variant of Henoch-Schönlein purpura (HSP) in the United States in 1913. Del Carril, Diaz Sobillo, and Vidal [2] described the condition in Argentina in 1936. Europeans have recognized Finkelstein's description of AHEI since his publication in 1938, [3] and, until recently, most reports of AHEI occurred in the European literature under the terms Finkelstein’s disease, Seidlmayer cockade purpura, [4] or purpura en cocarde avec oedema. Other names used for the disease include urticarial vasculitis of infancy and acute benign cutaneous leukocytoclastic vasculitis of infancy. [5]

More than 100 cases of AHEI have been published in medical literature worldwide. Although initially considered a variant of HSP, it is now considered a separate entity because of the infrequency of both visceral involvement and immunoglobulin A (IgA) skin depositions, [6, 5, 7, 8] as well a better prognosis. AHEI is characterized by the triad of fever; edema; and rosette-, annular-, or targetoid-shaped purpura primarily over the face, ears, and extremities in a nontoxic infant (see the image below). [9, 10, 11, 12] The cutaneous findings are dramatic both in appearance and rapidity of onset.

Large cockade (rosette or knot of ribbons), annula 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.

Although once thought to be a variant of HSP, AHEI is now considered a separate entity. 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.

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Pathophysiology

The specific etiology of acute hemorrhagic edema of infancy (AHEI) is unknown, although some consider the disease to be an immune complex–mediated vasculitis. [13] Eighty-four percent of reported cases were preceded by viral infections (acute upper respiratory tract infection, gastroenteritis), medication use (ie, antibiotics), and immunizations. [14, 15]

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Epidemiology

Frequency

United States

Acute hemorrhagic edema of infancy (AHEI) is uncommon in the United States. Specific frequency data have not been reported.

International

Acute hemorrhagic edema of infancy (AHEI) has been reported in countries throughout the world, although incidence is unknown.

Race

No racial predilection has been described for acute hemorrhagic edema of infancy (AHEI).

Sex

Acute hemorrhagic edema of infancy (AHEI) is more common among male infants than among female infants [16] ; the male-to-female ratio is approximately 4.6:1.

Age

The age range of onset for acute hemorrhagic edema of infancy (AHEI) is 2-60 months (median, 11 mo; mean, 13.75 mo). [16, 17] However, a congenital case of AHEI has been reported. [18]

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Prognosis

Acute hemorrhagic edema of infancy (AHEI) is usually benign, self-limited, and without sequelae, with spontaneous recovery occurring within 1-3 weeks. Rare reports have described complications such as arthritis, nephritis, [19, 20] abdominal pain, gastrointestinal tract bleeding, intussusception, [21] scrotal pain, and testicular torsion. [22] AHEI may recur, but this is uncommon. One case report describes an AHEI patient whose eruption resolved with unusual scarring. [15]

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Patient Education

Educate parents about the benign self-limited nature of acute hemorrhagic edema of infancy (AHEI) and the fact that recurrences, although uncommon, can occur.

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