Acute hemorrhagic edema of infancy-a rare entity

Abstract

A patient presenting with fever and purpura often presents a diagnostic challenge for the dermatologist. Acute hemorrhagic edema of infancy (AHEI) is a rare acute cutaneous leukocytoclastic vasculitic syndrome of infants leading to this presentation. We present an 18-month-old girl with AHEI who presented with fever, edema, and purpuric lesions involving the face, ears and extremities with uneventful complete recovery.

Acute hemorrhagic edema of infancy: a worrisome presentation, but benign course

“…The age group is typically between 4 and 24 months 1. In the majority of the cases, AHEI is a self-limited disease (1–3 weeks), but there have been reports of recurrent attacks (up to four attacks) 4…”

“…The skin changes are characterized as well demarcated, annular, medallion-like, rosette shaped purpuric plaques, and tend to appear suddenly in the face and extremities 1. However, the skin lesion does not always present in a typical way and it has been reported that it can appear as hemorrhagic blisters 3.…”

“…AHEI can also mimic urticarial vasculitis, but the former usually has an acute presentation with rare remissions 8. The presentation of AHEI can often resemble that of Henoch-Schönlein purpura (HSP) and some authors consider AHEI as a cutaneous variant of HSP 1. It is crucial to differentiate between AHEI and HSP since the latter requires systemic corticosteroid therapy.…”

“…It has also been described as Finkelstein’s disease, Seidlmayer’s disease, and postinfectious cockade purpura 1. AHEI is considered a benign phenomenon and it usually resolves spontaneously in a few weeks 2.…”

Acute Hemorrhagic Edema with extensive truck involvement and Rhinovirus infection association

“…Nonetheless, various associations have been reported such as infections that may play part of its pathogenesis (Fiore et al, 2008). Other associations have been reported like after using drugs or vaccinations (Freitas and Bygum, 2013;Findal and Kura, 2013). AHE has a male predominance and usually affects children less than 2 years of age.…”