Acute hemorrhagic edema of infancy: unusual scarring and review of the English language literature

Abstract

Acute hemorrhagic edema of infancy (AHEI) is a benign and self-limiting disease that usually resolves completely without sequelae and without the need for active therapy. Most consider AHEI to be a separate entity from Henoch-Schonlein purpura (HSP), but others believe that they are different clinical presentations of the same disease. We report a patient with AHEI who resolved with unusual scarring. We also present a review and update of AHEI synonyms, epidemiology, pathogenesis, clinical features, laboratory findings, histopathology, diagnosis and differentials, management, and prognosis. A comprehensive Medline search using the Pubmed search engine in the English-language literature, for relative articles addressing AHEI disease. Thirty-three articles were cited, in addition to our case report. Controversy still exists with regard to the separation of AHEI and HSP as distinct entities, as well as with regard to a conservative or active approach for AHEI treatment, and its effect on the course of the disease.