Describe a young adult with refractory red ear syndrome that responded to lidocaine.

Red ear syndrome (RES) is a rare condition first described by James W. Lance in 1994. Approximately 100 cases were reported so far. RES is defined as unilateral or bilateral paroxysmal painful erythema of the external ear lasting from seconds to hours. Episodes can occur spontaneously or triggered by rubbing or touching the ear, heat, chewing, brushing of the hair, or exertion. Early-onset RES tends to associate with migraine, whereas late-onset with trigeminal autonomic cephalgia. Secondary forms occur with C2 and C3 radiculopathy or temporomandibular joint dysfunction. Although most RES are refractory to medical treatment, some migraine-related attacks may benefit from migraine preventive therapies. Several peripheral and central nervous system mechanisms have been proposed but the pathophysiology remains unclear.

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We report a 21-year-old right-handed migrainous man who presented with red, hot and painful ears for the past 18 months. Pain was triggered by rubbing and accompanied by bilateral frontotemporal, sometimes retro-orbital, headache. Cold temperature or use of a fan alleviates the pain. Neurological examination was normal. MRI brain and cervical spine were negative. SCN9A and SCN11A gene mutations for Na channels were absent. Gabapentin, amitriptyline, duloxetine, oxcarbazepine, analgesic creams and patch provided minor relief. He responds well to monthly lidocaine infusions.

Our patient with refractory red ear syndrome resembling erythromelalgia responding well to lidocaine infusion. This suggests the possibility of using a sodium channel blocker, such as lidocaine infusion acutely, or oral mexiletine for maintenance. This case provides further insight into another management approach of this rare entity that may be confused with erythromelalgia.