Background::
Acute hemorrhagic edema of infancy (AHEI), a benign and self-limited disease, can be easily
mistaken to be a number of diseases with similar dermatological manifestations but with potentially adverse outcomes.
Objective:
This review aimed to familiarize pediatricians with the natural history, clinical manifestations, diagnosis, and
management of AHEI.
Methods:
A PubMed search was conducted in February 2020 in Clinical Queries using the key terms “acute hemorrhagic
edema of infancy” OR “Finkelstein disease” OR “Seidlmayer disease”. The search strategy included meta-analyses,
randomized controlled trials, clinical trials, observational studies, and reviews published within the past 10 years. Only
papers published in the English literature were included in this review. The information retrieved from the above search
was used in the compilation of the present article.
Results:
AHEI, a rare cutaneous leukocytoclastic small-vessel vasculitis, typically presents with palpable purpura,
peripheral acral edema, and frequently fever, most often in children between 4 and 24 months of age. A significant
number of children experience prodromal symptoms of an upper respiratory infection. Fever is typically low grade and is
present in approximately 50% of cases. The cutaneous lesions are characterized by rapid onset of small erythematous
macules or papules that progress to well demarcated, annular, rosette, medallion-like, or targetoid purpuric plaques or
ecchymosis in 24 to 48 hours. The skin lesions are typically palpable, nonpruritic, and symmetrically distributed. Sites of
predilection include the face, auricles, and extremities. Edema is typically nonpitting and asymmetrical and occurs
primarily on the dorsum of the hands and feet, the face, and the auricles. In spite of the acuteness and extent of the
cutaneous findings, the child looks well and nontoxic. Systemic and/or visceral involvement are rare. The differential
diagnosis is broad and includes, among others, Henoch-Schönlein purpura. It is crucial to distinguish AHEI from the other
diseases since the management of these diseases is quite different. The clinical features of mimickers of AHEI are
reviewed and clues to differentiate AHEI from these mimickers are highlighted. AHEI is a benign, self-limited disease
with complete spontaneous recovery in one to three weeks in the majority of cases.
Conclusion:
Recognizing this rare disease is important for the pediatrician to rapidly differentiate AHEI from other
potentially serious diseases that require prompt therapy and monitoring. With rapid recognition of AHEI, unnecessary
investigations and inappropriate interventions can be prevented and parental anxiety can be avoided.