Mormile Ilaria, Petraroli Angelica, Loffredo Stefania, Rossi Francesca Wanda, Mormile Mauro, Del Mastro Andrea, Spadaro Giuseppe, de Paulis Amato, Bova Maria
Department of Translational Medical Sciences and Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Via S. Pansini 5, 80131 Naples, Italy.
Institute of Experimental Endocrinology and Oncology "G. Salvatore" (IEOS), National Research Council (CNR), Via S. Pansini 5, 80131 Naples, Italy.
J Clin Med. 2021 Apr 1;10(7):1442. doi: 10.3390/jcm10071442.
Episodic angioedema with eosinophilia (EAE) (Gleich's syndrome) is a rare disease characterized by hypereosinophilia (up to 95 × 10 cells/L), recurrent episodes of angioedema, urticaria, weight gain, and fever, that occur at periodical intervals (usually every 3-4 weeks). The exact etiology of EAE is still unclear, but both eosinophils and abnormalities of cytokines homeostasis seem to play a pivotal role in the pathogenesis of the disease. In particular, the cyclic elevation of serum interleukin-5 before the increase in eosinophil count has been reported. Herein, we performed a broad literature review and report the case of a thirty-two-year-old woman with a two-year history of cyclic angioedema attacks, urticaria, periodic weight gain, and severe hypereosinophilia, diagnosed with EAE and treated with oral corticosteroids. Describing the most relevant clinical features of EAE reported so far in the literature, we aim to provide physicians with some useful tools to help them deal with this disease. In addition, we aim to raise awareness about this rare condition in which approved diagnostic classification criteria are currently missing.
伴有嗜酸性粒细胞增多的发作性血管性水肿（EAE）（格莱希综合征）是一种罕见疾病，其特征为嗜酸性粒细胞增多（高达95×10⁹个细胞/L）、反复发作的血管性水肿、荨麻疹、体重增加和发热，这些症状呈周期性发作（通常每3 - 4周发作一次）。EAE的确切病因仍不清楚，但嗜酸性粒细胞和细胞因子稳态异常似乎在该疾病的发病机制中起关键作用。特别是，有报道称在嗜酸性粒细胞计数增加之前血清白细胞介素 - 5会出现周期性升高。在此，我们进行了广泛的文献综述，并报告了一例32岁女性病例，该患者有两年周期性血管性水肿发作、荨麻疹、周期性体重增加和严重嗜酸性粒细胞增多的病史，被诊断为EAE并接受了口服糖皮质激素治疗。通过描述目前文献中报道的EAE最相关的临床特征，我们旨在为医生提供一些有用的工具，以帮助他们应对这种疾病。此外，我们旨在提高对这种目前尚无批准的诊断分类标准的罕见疾病的认识。
