Non-episodic angioedema with eosinophilia as a differential diagnosis of eosinophilia in young females

Background: Episodic angioedema with eosinophilia, also known as Gleich's syndrome, is a differential diagnosis in patients with recurrent angioedema with higher blood eosinophils. Meanwhile, less has been elucidated regarding non-episodic angioedema with eosinophilia (NEAE). This study aimed to examine the prevalence, clinical characteristics, and disease course of NEAE.

Methods: By reviewing the electronic medical records, we identified patients with NEAE among those referred to allergy clinics due to eosinophilia from January 2021 to December 2023 at Asan Medical Center.

Results: Among 687 patients with eosinophilia, 58 (8.4%) were diagnosed with and treated for NEAE. All patients were females, with a mean age of 31.79 years. The mean absolute blood eosinophil count was 4468.76 cells/μL. All patients reported symmetric angioedema of the lower legs, and 37 (63.8%) had additional angioedema of the upper arms. Twenty-five (43.1%) patients reported a preceding event prior to onset of angioedema. Systemic corticosteroids (mean total dose 1745 ± 508.49 mg) were prescribed to all patients, with a treatment duration of approximately 40 days to achieve resolution. Following the resolution of angioedema, 6 patients experienced persistent arthralgia, 1 developed chronic spontaneous urticaria, and 1 developed hypereosinophilic syndrome.

Conclusions: NEAE is an essential differential diagnosis in young female patients with eosinophilia, particularly those presenting with symmetric peripheral angioedema.