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

BACKGROUND: 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.

METHODS: 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.

RESULTS: 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.

CONCLUSION: 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.