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

非发作性血管性水肿伴嗜酸性粒细胞增多症是年轻女性嗜酸性粒细胞增多症的鉴别诊断之一

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Abstract

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.

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