The Red Puffy Ear Sign - A Clinical Sign to Diagnose a Rare Cause of Meningoencephalitis

Fortschritte der Neurologie-Psychiatrie (Jul 2012)

Prinz S, Dafotakis M, Schneider RK, Mühlenbruch G, Stopschinski BE 

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• azathioprine

We report on the case of a 63-year-old patient with a meningoencephalitis, presenting itself with headache and a general neuropsychological retardation. Additionally, a reddening and swelling of both auricles could be seen. Magnetic resonance imaging showed confluent, contrast-uptaking lesions. In the cerebrospinal fluid an aseptic lymphocytic pleocytosis was found. A biopsy of the ear revealed a chronic lymphoplasmacellular inflammatory reaction. We diagnosed a relapsing polychrondritis, an inflammatory disease of the cartilage, which can in rare cases affect the central nervous system. Typically, the patients complain about red swollen ears, the"red puffy ear sign". After initiation of steroid and azathioprin therapy the patient recovered fully.