Acute Hemorrhagic Edema of Infancy: A Diagnostic Challenge for the General Pediatrician

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.

Keywords: Acute hemorrhagic edema; Henoch-Schönlein purpura; leukocytoclastic vasculitis; palpable purpura; peripheralacral edema.