Toddler with lesions on cheeks, ears, and arms

THE CASE

Alarmed parents bring their healthy 14-month-old son to the office for evaluation of a rash that appeared on his face and arms 3 days ago. He had a fever and runny nose at that time, but the fever has since resolved and he is behaving normally.

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DERMCASE diagnosis  Acute hemorrhagic edema of infancy (AHEI)

Clinical findings

Acute hemorrhagic edema of infancy (AHEI) is a reactive, cutaneous, small vessel vasculitis that typically affects children in the first 3 years of life.^1,2 The classic triad includes fever and a viral prodrome followed by skin lesions and edema of limbs and face.^2,3 Cutaneous involvement is characterized by an abrupt onset of large erythematous patches on the cheeks, ears, and extremities.^4,5 Lesions evolve into purpuric plaques that can have scalloped borders and central clearing.^2,6 These plaques are often described as “rosette” purpura or “purpura en cockade.” The condition is usually asymptomatic but can occasionally be painful or pruritic.⁵ Children are usually well appearing.⁶

Pathogenesis

The etiology of AHEI is elusive, but 75% of patients have a history of immunization, infection (ie, upper respiratory infection, conjunctivitis, pharyngitis) or drug exposure 1 to 2 weeks prior to the onset of symptoms.^2,5 In the past, AHEI was considered to be an infantile variant of Henoch-Schönlein purpura (HSP) based on identical skin biopsy findings (leukocytoclastic vasculitis) and morphology of skin lesions, but it is now considered to be a separate entity.^1,4 Unlike HSP, AHEI does not consistently show immunoglobulin A deposition on immunofluorescence of skin biopsy specimens; it typically doesn't involve internal organs; and it has a benign self-limited course.^2,6

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Epidemiology

Acute hemorrhagic edema of infancy mainly affects infants aged between 4 and 24 months.² This disorder is often described as "relatively rare" with approximately 300 reported cases. However, it is believed to be frequently misdiagnosed and was previously considered a subtype of HSP, so the actual incidence is likely to be higher.

Differential diagnosis

In young children presenting with facial edema and urticarial lesions, urticaria multiforme should be considered.⁵ The differential diagnosis also includes erythema multiforme, urticaria, urticarial drug eruption, serum sickness-like reactions, Kawasaki disease, urticarial vasculitis, and Sweet syndrome.³ Patients presenting with large hemorrhagic lesions are sometimes evaluated for trauma, acute meningococcemia, and purpura fulminans.

Henoch-Schönlein purpura is usually at the top of the differential, and patients with features of both conditions have been reported.⁷ The young age of this patient (<3 years), the brief duration, and the focal nature in the skin each favor the diagnosis of AHEI.⁵

Prognosis

Acute hemorrhagic edema of infancy usually runs a short and benign course (1 to 3 weeks) and is typically followed by complete resolution of symptoms.^1,2,8 Relapses are infrequent.^2,5,6 Mucosal and visceral involvement are rare, although oral petechiae, conjunctival injection, abdominal pain, arthralgias, glomerulonephritis, and intussusception can occur.^5,9 Atrophic scarring on the extremities also has been reported.¹⁰ Cases of transient, mostly microscopic hematuria, transient proteinuria, and mild hypertension have been reported, but most of these findings could be attributed to an underlying infection.^1,3,4,6,11

Diagnosis and treatment

Identification of AHEI is a clinical diagnosis. Most laboratory findings are nonspecific. Leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate (ESR) and C-reactive protein may be present.¹⁰ Some clinicians will check a urinalysis to rule out any renal involvement, but the absolute necessity of this is debatable. Treatment is supportive. Antibiotics should be prescribed only for a concurrent bacterial infection. Antihistamines can be useful if the skin lesions are pruritic. Systemic corticosteroids have not been found to alter the natural course of the disease.⁵

The patient

The boy had a normal complete blood count, electrolytes, and ESR. Urinalysis was negative. He was able to feed normally, and the skin eruption waxed and waned for 3 more days before resolving without recurrence.

REFERENCES

1. Saraclar Y, Tinaztepe K, Adalioğlu G, Tuncer A. Acute hemorrhagic edema of infancy (AHEI)--a variant of Henoch-Schönlein purpura or a distinct clinical entity? J Allergy Clin Immunol. 1990;86(4 pt 1):473-483.

2. Paller AS, Mancini AJ. Vasculitic disorders. In: Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. 4th ed. Philadelphia, PA: Elsevier Saunders; 2011:483-496.

3. Caksen H, Odabaş D, Kösem M, et al. Report of eight infants with acute infantile hemorrhagic edema and review of the literature. J Dermatol. 2002;29(5):290-295.

4. Gonggryp LA, Todd G. Acute hemorrhagic edema of childhood (AHE). Pediatr Dermatol. 1998;15(2):91-96.

5. Shinkai K, Fox LP. Cutaneous vasculitis. In: Bolognia JL, Jorizzo JL, Schaffer JV (eds). Dermatology. 3rd ed. Elsevier Saunders; 2012:385-410.

6. Legrain V, Lejean S, Taïeb A, Guillard JM, Battin J, Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol. 1991;24(1):17-22.

7. Di Lernia V, Lombardi M, Lo Scocco G. Infantile acute hemorrhagic edema and rotavirus infection. Pediatr Dermatol. 2004;21(5):548-550.

8. Alhammadi AH, Adel A, Hendaus MA. Acute hemorrhagic edema of infancy: a worrisome presentation, but benign course. Clin Cosmet Investig Dermatol. 2013;6:197-199.

9. Watanabe T, Sato Y. Renal involvement and hypocomplementemia in a patient with acute hemorrhagic edema of infancy. Pediatr Nephrol. 2007;22(11):1979-1981.

10. AlSufyani MA. Acute hemorrhagic edema of infancy: unusual scarring and review of the English language literature. Int J Dermatol. 2009;48(6):617-622.

11. Amitai Y, Gillis D, Wasserman D, Kochman RH. Henoch-Schönlein purpura in infants. Pediatrics. 1993;92(6):865-867.

Dr Vlassova is a fourth-year resident, Department of Dermatology, University of Pittsburgh Medical Center (UPMC), Pennsylvania. Dr Gehris is chief, Pediatric Dermatology, and medical director, Pediatric Teledermatology, Children’s Hospital of Pittsburgh of UPMC, Pennsylvania. Dr Cohen, section editor for Dermcase, is professor of pediatrics and dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland. The authors and section editor have nothing to disclose in regard to affiliations with or financial interests in any organizations that may have an interest in any part of this article. Vignettes are based on real cases that have been modified to allow the authors and section editor to focus on key teaching points. Images also may be edited or substituted for teaching purposes.