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Red Ear Syndrome: a Case Presentation and Discussion Author Affiliations Are Listed at the End of This Article Brackenrich et al. HCA Healthcare Journal of Medicine (2020) 1:3 https://doi.org/10.36518/2689-0216.1053 Case Report Red Ear Syndrome: A Case Presentation and Discussion Author affiliations are listed at the end of this article. Jared Brackenrich, DO,1 Samantha Sternad, DO,1 Chad Johnston, DO2 Correspondence to: Abstract Jared Brackenrich, DO Introduction LewisGale Hospital - Mont- Red Ear Syndrome (RES) is a condition often considered to be a localized form of erythro- gomery melalgia. It can be related to structural cervical defects or idiopathic. RES is generally very 3700 S Main St difficult to treat. Blacksburg, VA 24060 ([email protected]) Discussion A 57-year-old male presented to the dermatology clinic complaining of a 4-month history of intermittent redness and severe burning of bilateral ears. On examination, the patient exhib- ited edematous erythema and tenderness to palpation affecting the right and left ear and right malar cheek. A skin biopsy revealed mild superficial perivascular lymphocytic infiltrate with hypertrophy of endothelial cells. The patient was found to have a normal lab work-up including complete blood count, metabolic panel, erythrocyte sedimentation rate, anti-nu- clear antibody and type II collagen antibody. A diagnosis of Red Ear Syndrome was made. After failing multiple medications over several months, the patient was started on aspirin and paroxetine which was gradually titrated until he was completely asymptomatic. To date, there is only one other case presentation illustrating the effectiveness of this treatment regimen. Conclusion There are a limited number of cases describing idiopathic RES with inconsistent results in treatment. With a relatively small number of cases reported, further research is needed into the pathophysiology of RES along with the dual therapy of aspirin and paroxetine in patients that suffer from both primary and secondary RES. Keywords red ear syndrome; pain/diagnosis; syndrome; erythema/physiopathology; ear diseases; erythromelalgia; paroxetine; aspirin Case Presentation carbamazepine, brimonidine, amitriptyline, pro- A 57-year-old male presented to the dermatol- pranolol, labetalol and verapamil. The patient ogy clinic with a 4-month history of intermit- was notably frustrated given the impact this tent, severe redness and burning that started had on his physical and mental health. on his right ear and progressed to his right cheek and left ear. The episodes would last Physical examination revealed significant ery- several hours but, in some cases, persisted for thema, swelling and pain with palpation of the days. The redness and pain worsened with sun right and left external ear and partial right ma- exposure and overheating. Conversely, cool lar cheek. (Figure 1) Given the patient’s insecu- weather and cold compresses seemed to allevi- rities with the redness, he was wearing makeup ate his symptoms temporarily. The patient had daily to cover this. Both earlobes were notably already been established with an otolaryngolo- involved. On subsequent visits, the redness and gist for this issue and had failed prior therapies pain began affecting bilateral hands. including gabapentin, pregabalin, imipramine, We first obtained punch biopsies from skin of www.hcahealthcarejournal.com HCA Healthcare © 2020 HCA Physician Services, Inc. d/b/a Journal of Medicine Emerald Medical Education 155 HCA Healthcare Journal of Medicine Figure 1. Erythema and swelling of the right external ear and right malar cheek observed after wiping away makeup. the right posterior ear and right superior malar mexiletine and nerve blocks, which only provid- cheek, revealing mild superficial perivascular ed minimal relief. After months of poor ther- lymphocytic infiltrate with mild hypertrophy of apeutic response, the patient was referred to endothelial cells. (Figures 2 and 3) The histology neurosurgery, but further medical treatment revealed no increase in mast cells. While the was recommended before any surgical inter- biopsy results were pending, a complete blood vention. count, metabolic panel, erythrocyte sedimen- tation rate, anti-nuclear antibody and type II Almost one year after the patient first de- collagen antibody were obtained and found to veloped symptoms and multiple treatment be within normal limits. Based on the patient’s regimens, he was started on paroxetine 10 mg, clinical, histologic and laboratory findings, a while continuing aspirin 325 mg twice daily. The diagnosis of Red Ear Syndrome (RES) with dose of paroxetine was gradually increased up erythromelalgia of the hands was made. Brain to 80 mg daily over the course of three months and cervical spine magnetic resonance imaging until the patient’s symptoms resolved. (MRI) were performed to look for structural neurologic defects, but only mild age-appro- Discussion priate degenerative changes were observed at RES is a rare condition that is characterized by C4-5. episodic erythema of the ear accompanied by a burning sensation or otalgia. Both ears, or more Additional therapies based on prior case re- commonly one ear, may be affected, and the ports were attempted including topical an- syndrome can extend to the face. Symptoms esthetics, aspirin, indomethacin, duloxetine, Figure 2. Histopathology of Red Ear Syndrome. Punch biopsy revealed mild superficial perivascu- lar lymphocytic infiltrate and hypertrophy of endothelial cells. (H&E, original magnification x10) 156 Brackenrich et al. (2020) 1:3. https://doi.org/10.36518/2689-0216.1053 Figure 3. Histopathology of Red Ear Syndrome. Punch biopsy revealed mild superficial perivascu- lar lymphocytic infiltrate and hypertrophy of endothelial cells (Arrows). (H&E, original magnifica- tion x20) can be spontaneous or triggered by touch, substances. A central mechanism has also been exertion, heat or cold, stress and even dietary proposed, regarding the connection between triggers.1, 2 RES has a predilection for females the trigeminal nerve and facial parasympathet- with a mean age of affliction of 40.2 years.3 ic outflow, especially for those patients that Only 47 cases of men suffering from RES had experience migraines.10, 11 Lastly, local damage to been reported prior to a 2013 study.4 small nerve fibers and vessels as seen in eryth- romelalgia has been a possible explanation.8, 10 RES was first described by Lance in 1994 as a One of the most common conditions included neurological syndrome presenting with flush- in the differential of RES is erythromelalgia, ing, tightness and pain in one ear lobe.5 He which is a rare autosomal dominant neuropa- later distinguished between primary (either thy, characterized by a combination of recur- isolated or associated with headache attacks) rent burning pain, warmth and redness of the and secondary cases.6 Primary RES is an id- hands and feet. Pain and redness are usually iopathic form that more commonly occurs in bilateral. Patients have reported involvement adolescents and is typically associated with of the face but there is controversy over the headaches or migraines. This type is less likely possibility that this denotes a unique pain syn- to be triggered by stimulation.3 The secondary drome rather than RES itself.12-14 RES is com- form more frequently affects adults and has monly considered an auricular version of eryth- been attributed to a wide range of illnesses romelalgia.15 Histology is similar between the including cervical spondylosis, traction injury of two conditions with both showing perivascular upper cervical roots, atypical glossopharyngeal/ lymphocytic inflammation, perivascular edema trigeminal neuralgias, TMJ dysfunction and and arteriolar smooth muscle hyperplasia.13 RES thalamic syndrome.5, 7-10 can clinically be distinguished from its erythro- melalgia counterpart by the classic presenta- Diagnostic criteria have been proposed for tion affecting solely the external ear. RES by Lambru.4 Criteria require at least 20 attacks of ear pain accompanied by redness Due to the association of secondary RES with lasting up to four hours. These attacks general- upper cervical spine pathology, an MRI of the ly occur more than once daily and have at least cervical spine should be carried out when RES two of the following characteristics: burning is suspected. In addition, if TMJ dysfunction is pain, unilateral, mild to moderate severity and a possibility, an orthodontic assessment is war- triggering by cutaneous or thermal stimulation ranted. Lastly, an MRI scan of the brain should of the ear.4, 10 The pathophysiology of RES is un- be included to rule out thalamic lesions.4 If skin clear, but many theories have been postulated. involvement is more generalized, a complete In the case of secondary RES, irritation of the blood count and thermograph should be com- C3 nerve root has been suggested.5 This nerve pleted to rule out other secondary causes.16 innervates the skin of the ear causing a pain RES is generally considered refractory to treat- sensation through the release of vasodilating ment and most cases show multiple trials of 157 HCA Healthcare Journal of Medicine medications with little to no relief. Medications expressed in this publication represent those of that have been shown to be at least partially the author(s) and do not necessarily represent effective for primary RES include gabapentin, the official views of HCA Healthcare or any of amitriptyline, paroxetine, indomethacin, ibupro- its affiliated entities. fen, aspirin, flunarizine, nimodipine, pregabalin 3, 4 and a greater auricular nerve blockade. Sec- Author
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