by Kristy Fleming, MD
An otherwise healthy 10-month-old boy presented to the emergency department (ED) with a rapidly progressing rash. His grandmother noted that the child had suffered for a week from a runny nose, mild fatigue and subjective fever. The child’s pediatrician saw him two days prior to presentation at the ED, and initiated amoxicillin. The morning after starting the antibiotic, the patient began developing swelling in his hands, as well as erythematous edematous medallion-like purpuric papules and plaques on the face, ears, arms, dorsal hands and legs. The baby appeared uncomfortable but was awake, alert and appropriate on exam. What’s your diagnosis?Submit your answer, and then read the full explanation below. If you like this activity or have a suggestion, tell us about it in the comment box at the bottom of the page.
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Acute hemorrhagic edema of infancy (AHEI) is also known as Finkelstein’s disease, Seidlmayer cockade purpura, medallion-like purpura, purpura en cocarde avec oedema, and infantile post-infectious iris-like purpura and edema.1 AHEI is a rare form of cutaneous small-vessel vasculitis that typically affects children younger than 24 months and is more predominant in boys.1,2
Approximately 75% of children present complaining of a recent upper respiratory infection, antibiotic exposure and/or immunization.1,2 Numerous infectious agents have been implicated as possible triggers, including: staphylococci, streptococci, adenovirus, Escherichia coli, coxsackievirus and rotavirus.1 The pathophysiology is thought to involve immune-complex deposition in small cutaneous vessels.
Patients often present with a low-grade fever, but otherwise non-toxic appearance. Internal involvement, such as joint pains, gastrointestinal symptoms and renal involvement is rare.2 Mild facial and acral edema is common.
The cutaneous eruption is abrupt and progresses quickly over the course of one to two days. Lesions are edematous, erythematous, purpuric plaques and petechial, and have been described in the literature as medallion-like, rosette-shaped and cockade appearing. The lesions may be mildly tender and favor the face, ears, extremities and occasionally the scrotum.3
The morphologic appearance can sometimes be confused with the targetoid lesions of erythema multiforme or Stevens-Johnson syndrome (SJS). However, the distribution and lack of internal involvement help to differentiate AHEI from these disorders.
AHEI can be distinguished from Henoch-Schölein purpura (HSP) by several characteristics: age of onset younger than 2 years, lack of systemic symptoms, and a briefer duration with spontaneous resolution.2
Clinicians can generally suspect an AHEI diagnosis based on clinical presentation and thorough history. Ruling out more serious disorders such as SJS, HSP and meningococcemia is most important.
Routine laboratory tests in AHEI are unremarkable. Mild leukocytosis occurs on occasion, but without hematuria, proteinuria or hematochezia.3 A skin biopsy may be performed if the diagnosis is questionable and will show leukocytoclastic vasculitis of the capillaries and post-capillary venules in the upper and mid dermis.1
Clinicians may also wish to perform direct immunofluorescence (DIF) to help exclude HSP, which shows immunoglobulin A deposition in vessel walls; however, approximately 30% of AHEI cases may also have IgA vascular deposits, so DIF alone cannot be used as a discriminating test.1
AHEI is a self-limited disease; therefore, supportive treatment alone is sufficient. Antibiotics should be prescribed to treat any associated bacterial infections. Otherwise, spontaneous recovery without scarring and without sequelae occurs within 12 to 20 days.1,2 Recurrent episodes are rare, as are complications, which may include arthritis, nephritis, abdominal pain, gastrointestinal tract bleeding, intussusception, scrotal pain or testicular torsion
If there is significant abdominal or renal involvement, the patient should be referred to a gastroenterologist or nephrologist.
1. J Bolognia, JL Jorizzo, RP Rapini. Dermatology. 2nd Ed. Elsevier; 2008: 353-354.
2. James WD, Berger TG, Elston DM. Andrew’s Diseases of the Skin Clinical Dermatology. 10th ed. Pennsylvania: Saunders Elsevier; 2006: 833-834.
3. Paller AS, Mancini AJ. Hurwitz Clinical Pediatric Dermatology. 3rd Ed. Elsevier; 2006:560-561.