Red ear syndrome in children: Review of literature and report of three cases *

Background: 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. Conclusion: 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.