Acute Hemorrhagic Edema of Infancy: A Safe Diagnosis or a Diagnostic Challenge? A 15 Year Retrospective Analysis

BACKGROUND: Acute hemorrhagic edema of infancy (AHEI, or Finkelstein-Seidlmayer vasculitis) is a benign, self-limiting small vessel vasculitis that is typically diagnosed through clinical assessment alone. Its clinical overlap with diseases that have potentially more complications, such as IgA vasculitis (IgAV), poses diagnostic challenges, balancing the risk of over-treatment against the potential for missed or delayed diagnoses. This study aims to describe clinical experience with diagnosing AHEI and to identify factors associated with a diagnostic revision to IgAV. METHODS: This large Dutch retrospective cohort study analyzed 89 children clinically diagnosed with AHEI between 2007 and 2024. Demographic, clinical, and laboratory data were collected from medical records. A revised diagnosis was defined as a revision of the initial AHEI diagnosis during follow-up. Descriptive analysis and logistic regression were performed to determine factors associated with diagnostic revisions. RESULTS: Of 89 patients included, 20 (22.5%) had their initial diagnosis revised, with 9 cases reclassified as IgAV. Risk factors for the diagnostic revision to IgAV included older age (median 37 vs. 15 months, p < 0.001), presence of a painful rash (33% vs. 10%, p = 0.044), and absence of blanchable lesions (22.2% vs. 76.2%, p < 0.001). Complications occurred in seven of nine patients (78%) with IgAV. CONCLUSIONS: This study describes the AHEI patient population and highlights the importance of age, rash characteristics, and clinical features in differentiating AHEI from potentially more severe conditions such as IgAV. A proposed clinical flowchart including patient age and rash characteristics may improve diagnostic accuracy and optimize patient follow-up, balancing the need for vigilance while avoiding unnecessary treatment.