Acute hemorrhagic edema of infancy: 20-year experience from an Italian tertiary referral center

Purpose: Data on acute hemorrhagic edema of infancy (AHEI) are derived from small case series or case reports. We report a 20-year experience at a national referral center.

Methods: We performed a single-center retrospective study including patients who were diagnosed with AHEI from January 1, 2004, to June 30, 2023.

Results: We identified 21 patients (57.1% females) with a median age of 18 months (range 7-33 months). Thirteen (61.9%) patients were admitted to the pediatric ward, the remaining eight (38.1%) presented to the emergency department and were discharged for outpatient management. The median length of hospitalization was 5 days (range 3-9 days). Twenty patients (95.2%) had prodromal symptoms. The most common cutaneous findings were targetoid purpuric plaques. The lesions were most localized on the face (13, 61.9%) and on the upper limbs (18 patients, 85.7%). Sixteen (76%) patients presented with nonpitting and tender edema, localized on the feet (9/16, 56%) and hands (6/16, 37.5%). Systemic involvement was rare, and no patients experienced complications or sequelae. Twelve (57.1%) patients underwent infectious disease investigations, with positive results in only four (33.3%). None of the patients diagnosed after the SARS-CoV-2 outbreak (March 2020) had positive nasopharyngeal swabs for the virus. For the 13 patients who were admitted to the pediatric ward, the median length of hospitalization was five days (3-9 days).

Conclusions: The 21-patient single-center cohort of children affected by AHEI confirmed a generally benign course of AHEI, despite a 62% rate of hospitalization.