A refractory case of erythromelalgia involving the ears

Ramirez, Claudia C.; Kirsner, Robert S.

doi:10.1016/j.amjoto.2004.02.008

Cited by 17 publications

(30 citation statements)

References 11 publications

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“…It can radiate towards the cheek and mandible [16,20], as well as towards the occiput [4] and the involvement of the V1 area or whole hemicranium has also been described [1,10,12]. …”

Section: Reviewmentioning

confidence: 99%

“…However, some patients can exhibit exclusively spontaneous attacks (25 patients) [5,9,14] or triggered attacks (14 patients) [2,7,11]. Triggers most often include: heat (reported in six patients) [2,10,15,19,20], rubbing the ear (three patients) [7,15,23], physical exercise (three patients) [2,9,20] and neck movement (three patients) [2,7]. Other cutaneous stimulations able to provoke RE episodes include: light touching of the ear, brushing of the hair, chewing, tooth grinding and showering [2,10,16].…”

Section: Reviewmentioning

confidence: 99%

“…Two patients failed topical application of lidocaine [12,20] and two patients topical steroids (clobetasol 0.05% cream being used in one such case) [15,20]. However, cooling of the ear by application of an ice-pack has been reported to provide significant relief in five patients during an acute attack [2,5,15,19].…”

Section: Reviewmentioning

confidence: 99%

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The red ear syndrome

2013

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Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed.

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“…It can radiate towards the cheek and mandible [16,20], as well as towards the occiput [4] and the involvement of the V1 area or whole hemicranium has also been described [1,10,12]. …”

Section: Reviewmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

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The red ear syndrome

2013

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“…Its symptoms may be triggered by warmth or moderate exercise, and can be prevented or relieved by cooling [1-6]. It primarily affects the extremities, particularly the hands and the feet, and may infrequently extend to other parts of the body including the neck, face, ears, nose and scrotum [1,2,7].…”

Section: Introductionmentioning

confidence: 99%

“…It primarily affects the extremities, particularly the hands and the feet, and may infrequently extend to other parts of the body including the neck, face, ears, nose and scrotum [1,2,7]. In rare instances, localized entity on a thigh [3]; sole involvement on vulva [4], on cheeks [5] and on ears [6] have also been described in the literature. EM includes primary and secondary forms.…”

Section: Introductionmentioning

confidence: 99%

Erythema associated with pain and warmth on face and ears: a variant of erythermalgia or red ear syndrome?

Chen

Luo

et al. 2014

J Headache Pain

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Erythermalgia is a rare cutaneous disorder characterized by attacking of erythema, pain and increased temperature, which primarily involves the extremities and may infrequently extend to the neck, face, ears and even the scrotum. We reported an 18-year-old woman who presented with 3 years history of sole involvement of attacking erythema, pain and warmth over her face and ears without any other associations. The frequency and severity of the flares progressed gradually during the course. Cutaneous examination revealed erythema, increased temperature and tenderness on the face and ears during the flare. The symptoms could be relieved rapidly by cooling. Dermatoscope showed that vessels inside the erythema were more dilated during the episode than after application of ice. The lesion is considered a rare variant of erythermalgia with sole involvement of face and ears. The symptoms had mild response to oral antihistamines, topical steroids and tacrolimus, but had excellent response to the combinative therapy of aspirin and paroxetins.

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Red Ear Syndrome

Raieli

Compagno²,

D’Amelio

2016

Curr Pain Headache Rep

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The Red Ear syndrome (RES) is an intriguing syndrome originally described for the first time nearly 20 years ago. RES is characterized by unilateral/bilateral episodes of pain and burning sensation of the ear, associated with ipsilateral erythema. RES episodes are indeed isolated in some patients, but they can occur in association with primary headaches, including in particular migraine in the developmental age. Although the underlying pathophysiological mechanisms are still uncertain, in the recent years the described comorbidities have aroused increasing interest because of possible clinical implications. Moreover, RES seems to be more often associated with clinical features of migraine partially provoked by the involvement of the parasympathetic system. This clinical association has shed new light on the pathophysiology of RES, supporting the hypothesis of a shared pathophysiological background, for example, through the activation of the trigeminal autonomic reflex. Current therapies of RES will be also discussed. Finally, we will resume the more controversial aspects of this relatively new and probably underestimated neurological syndrome.

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A refractory case of erythromelalgia involving the ears

Cited by 17 publications

References 11 publications

The red ear syndrome

The red ear syndrome

Erythema associated with pain and warmth on face and ears: a variant of erythermalgia or red ear syndrome?

Red Ear Syndrome

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