Child Neuropsychiatry School - University of Palermo, Piazzale Delle Cliniche Palermo, 90100, Italy.
Child Neuropsychiatry Unit- ISMEP -P.O. Cristina - ARNAS Civico, Via Dei Benedettini 1Palermo, 90100, Italy.
Int J Pediatr Otorhinolaryngol. 2021 Mar;142:110615. doi: 10.1016/j.ijporl.2021.110615. Epub 2021 Jan 6.
Red ear syndrome (RES) is a neurological syndrome that is characterized by attacks of redness and pain that is localized in the earlobe, accompanied by a burning sensation, swelling or otalgia. The exact pathophysiology of RES is not known. Several pediatric cases have been described. They show an extreme variability in clinical presentation and therapeutic response, and therefore there are numerous difficulties in the diagnostic-therapeutic approach and in the comprehension of the physiopathology. The goal of this report is to present three clinical cases of red ear syndrome in children. These cases show various characteristics that can give useful indications regarding the differential diagnosis and the pathogenetic mechanisms that are involved, particularly when they are compared with cases published in the literature.
CASE-REPORTS: We report three pediatric RES cases: 1) a boy whose condition offered a typical example of the association that occurs between migraine and RES. 2) a girl with idiopathic RES. 3) a child who suffered RES attacks that showed many similarities with trigeminal autonomic cephalalgias.
Our clinical series shows the different ways in which RES can be expressed and they support the reported scientific literature. We suggest that the different forms of RES have a common final autonomic pathogenetic mechanism that is activated by parasympathetic hyperactivity and sympathetic inhibition. The different temporal characteristics, frequency, etc. may depend on the activation of distinct physiopathological modules that are related to the pain circuits, as suggested by the modular theory which describes that groups of neurons are defined as a module, where each module is responsible for a symptom and the individual's headache is defined by the activated modules.
红耳综合征（RES）是一种以耳部发红和疼痛为特征的神经系统综合征，局限于耳廓，伴有烧灼感、肿胀或耳痛。RES 的确切病理生理学机制尚不清楚。已经描述了一些儿科病例。它们在临床表现和治疗反应方面表现出极大的可变性，因此在诊断-治疗方法和对病理生理学的理解方面存在许多困难。本报告的目的是介绍儿童红耳综合征的三个临床病例。这些病例显示出各种特征，这些特征可以为鉴别诊断和所涉及的发病机制提供有用的指示，特别是与文献中报道的病例进行比较时。
我们报告了三个儿科 RES 病例：1）一个男孩，他的情况典型地说明了偏头痛和 RES 之间发生的关联。2）一个患有特发性 RES 的女孩。3）一个患有 RES 发作的孩子，其发作与三叉自主头痛非常相似。
我们的临床系列显示了 RES 可以表现出的不同方式，并支持已报道的科学文献。我们认为，不同形式的 RES 具有共同的最终自主发病机制，该机制由副交感神经亢进和交感神经抑制激活。不同的时间特征、频率等可能取决于与疼痛回路相关的不同生理病理模块的激活，这正如描述了神经元群被定义为一个模块的模块化理论所表明的那样，其中每个模块负责一个症状，而个体的头痛则由激活的模块定义。
