Acute Hemorrhagic Edema of Infancy

AHEI

Ceci M, et al. Acute Hemorrhagic Edema of Infancy: Still a Challenge for the Pediatrician. Pediatr Emerg Care. 2016 May 30. [Epub ahead of print]

We report a case of acute hemorrhagic edema of infancy (AHEI) occurred in an 11-month-old male infant after upper respiratory tract infection. The onset was dramatic with petechiae, ecchymosis, and annular, nummular, or targetoid purpuric plaques on the extremities, face, and ears. Acute hemorrhagic edema of infancy is a benign form of leukocytoclastic vasculitis that typically affects children between 4 and 24 months of age. The etiology remains still unknown. The potential triggers of AHEI include preceding bacterial or viral infections, immunizations, and drugs. Although the clinical picture is fearful, in the majority of cases, it involves only cutaneous small vessels. Recognizing this as a distinct clinical entity allows to establish an appropriate prognosis for this rare benign disease in children.This report could be a helpful reminder, especially for emergency physicians, to discriminate AHEI from other more severe diseases, such as meningococcal sepsis.

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Serra E Moura Garcia C, et al. Acute Hemorrhagic Edema of Infancy. Eur Ann Allergy Clin Immunol. 2016 Jan;48(1):22-6.

Acute Hemorrhagic Edema of Infancy is a small vessel leucocytoclastic vasculitis affecting young infants. It is characterized by large, target-like, macular to purpuric plaques predominantly affecting the face, ear lobes and extremities. Non-pitting edema of the distal extremities and low-grade fever may also be present. Extra-cutaneous involvement is very rare. Although the lesions have a dramatic onset in a twenty-four to forty-eight hour period, usually the child has a non-toxic appearance. In most cases there are no changes in laboratory parameters. The cutaneous biopsy reveals an inflammatory perivascular infiltrate. It is a benign and auto-limited disease, with complete resolution within two to three weeks leaving no sequelae in the majority of cases. No recurrences are described. We report a case of a 42-day old girl admitted at our hospital with Acute Hemorrhagic Edema of Infancy.

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Glamann JA, et al. On target: a case of acute hemorrhagic edema of infancy. Hosp Pediatr. 2014 Mar;4(2):106-8.

“AHEI is an uncommon, self-limiting acute cutaneous vasculitis that was first described by Snow in 1913. [1] Nearly 100 years later, Blasini et al [2] described AHEI as a rare, benign acute cutaneous vasculitis that occurs between 4 and 24 months of age. The authors concur with Snow that the disease is self-limiting and further characterize it as edematous purpuric plaques on the extremities, the acute appearance of edema, and fever. AHEI is more commonly reported in the winter season, and illness duration is 5 to 35 days. [3,4] Bacterial or viral infections, medications, and immunizations have all been recognized as possible contributors to the onset of AHEI. [4,5] It is reported in all areas of the world with no racial predominance.”

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Savino F, et al. Acute hemorrhagic edema of infancy: a troubling cutaneous presentation with a self-limiting course. Pediatr Dermatol. 2013 Nov-Dec;30(6):e149-52.

Acute hemorrhagic edema of infancy (AHEI) is an unusual form of leukocytoclastic vasculitis with dramatic distinguishing skin lesions that occurs in infants ages 4 to 24 months old. The disease presents with skin eruptions that usually start with large (1-5 cm), symmetrically distributed, hemorrhagic lesions in a characteristic cockade pattern. The lesions are typically located on the lower extremities, face (in particular the ears, cheeks, and eyelids), and gluteal area. Fever may accompany skin eruptions. Clinical presentation at onset requires clinical and laboratory examination to distinguish it from more serious diseases and other vasculitis. The main differential diagnosis of AHEI is Henoch-Schönlein purpura. AHEI is generally a self-limiting disease, so a conservative approach should be considered. Topical or systemic corticosteroid therapy has been reported to be beneficial, as well as antihistamines and dapsone, although AHEI usually resolves completely with or without treatment. We report two cases of AHEI and an update of the literature.

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More PubMed results on AHEI.

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