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Acute hemorrhagic edema of infancy (AHEI) is characterized by the rapid onset of edema in association with a cockade pattern of purpuric lesions initially located on the extremities that affects children under the age of 2 years. The disease usually follows a benign course and there is no visceral involvement. Diagnosis is clinical, since laboratory tests are nondiagnostic and histologic analysis of the lesions (which is unnecessary in the majority of cases) is usually nonspecific (occasionally with features of nonspecific leukocytoclastic vasculitis). Its classification remains uncertain and some authors believe it to be a clinical variant of Henoch-Schonlein purpura (as attested by the simultaneous observation of Henoch-Schonlein purpura and AHEI in a brother and sister).
It is therefore the rapid onset of cockade purpuric lesions or pseudo-necrotic plaques and edema in an otherwise healthy child that points to the diagnosis, sometimes with an antecedent upper respiratory tract infection in the previous 8 to 10 days. There is a stark contrast between the profuse, dramatic appearance of the lesions and the patient’s good general state of health. The most common differential diagnosis is purpura fulminans. The child should be closely monitored in the first few days after onset although complications are extremely rare (anecdotal reports of acute intestinal invagination?).
Spontaneous recovery usually occurs in about ten days. Therapeutic management consists of close surveillance of the child’s general state of health. Fever, enlargement of the purpuric lesions and above all, a change in general state of health may be a harbinger of purpura fulminans. If there is the slightest doubt, additional tests (lumbar puncture in particular) should be ordered to rule this out, and the child should be hospitalized with systemic antibiotic therapy while awaiting the lumbar puncture results. Another common differential diagnosis (which is non-serious) is acute hemorrhagic or ecchymotic urticaria, but in this case the lesions are more ecchymotic and they coexist or follow true labile, pruritic urticarial lesions, two features which help avoid confusion between the two entities.
No treatment is necessary, apart from antipyretics if the patient is febrile. The skin lesions, which may be quite dramatic, disappear without scarring and require no specific treatment.
A previous viral infection has been suggested to be a trigger of AHEI but this remains to be demonstrated.
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