Red ear syndrome--chronic pain syndrome of low prevalence.

A case is described of a 72-year-old woman that presented to our outpatient neurology clinic complaining of relapsing attacks of severe pain in and around the left ear and ipsilateral side of the face, accompanied by objectively visible cutaneous erythema with burning sensation throughout the attack. There was no alteration in her neurologic status. Comprehensive tests were done to exclude the possible primary diseases. The findings were within the limits of physiological values. Attempts at treating the pain with various analgesics and a combination of analgesics and first generation anticonvulsants did not prove effective. The pain was slightly relieved with pregabalin in maximum dosage. Because of various potential pathophysiological mechanisms, literature does not indicate any unique potential treatment approach, and some patients are resistant to any kind of therapy.