Acute Hemorrhagic Edema of Infancy

Updated: May 18, 2018
  • Author: Donald Shenenberger, MD, FAAD, FAAFP; Chief Editor: William D James, MD  more...
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First described in 1913 by Snow, [1] acute hemorrhagic edema of infancy (AHEI) was originally thought to be a purely cutaneous variant of Henoch-Schönlein purpura (HSP). Over the years, many others have described AHEI, including Del Carril, Diaz Sobillo, and Vidal [2] in Argentina in 1936 and Finkelstein in Europe 1938. [3] AHEI has also continued to be known by other names, including Seidlmayer cockade purpura, [4] purpura en cocarde avec oedema, urticarial vasculitis of infancy, and acute benign cutaneous leukocytoclastic vasculitis of infancy. [5]

To date, approximately 300 cases of AHEI have been published in medical literature worldwide. [6] AHEI is now considered a separate entity from HSP because of the infrequency of both visceral involvement and immunoglobulin A (IgA) skin depositions, [7, 5, 8, 9] 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 or young child (see the image below). [10, 11, 12, 13] 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.

HSP, in comparison, usually presents with palpable purpura or petechiae associated with one 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.



The specific etiology of acute hemorrhagic edema of infancy (AHEI) is unknown, although some consider the disease to be an immune complex–mediated vasculitis. [14] Eighty-four percent of reported cases were preceded by viral infections (acute upper respiratory tract infection, gastroenteritis), medication use (ie, antibiotics), and immunizations. [15, 16] In one review of a large referral center in Israel, various infectious conditions were identified in patients with AHEI, including Escherichia coli urinary tract infection, rota virus gastroenteritis, adenovirus upper respiratory tract infection, Streptococcus pyogenes tonsillitis, and herpes simplex gingivostomatitis. [17] In addition, reports have also described an association with Coxsackie virus infection. [18, 19]



The cause of acute hemorrhagic edema of infancy (AHEI) is unknown. AHEI is considered to be an immune complex disorder; however, immune complexes have been demonstrated in only some cases. [6, 20]




United States

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


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


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


Acute hemorrhagic edema of infancy (AHEI) is more common among male infants than among female infants [6] ; the male-to-female ratio is approximately 4.6:1.


The age range of onset for acute hemorrhagic edema of infancy (AHEI) is 2-60 months (median, 11 mo; mean, 13.75 mo). [6, 21] However, a congenital case of AHEI has been reported. [22]



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, [23, 24] abdominal pain, gastrointestinal tract bleeding, intussusception, [25] scrotal pain, and testicular torsion. [26] AHEI may recur, but this is uncommon. One case report describes an AHEI patient whose eruption resolved with unusual scarring. [16]   


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.