A Case of Relapsing Polychondritis with Facial Nerve Palsy

Abstract

Relapsing polychondritis (RP) is a rare systemic disease that leads to recurrent chondritis affecting the ear, nose, throat, and trachea. RP rarely presents with neurological symptoms, in only up to 3% of patients. A case of an 84-year-old woman who presented with left facial nerve palsy, left auricular swelling, and fever is reported. She was initially diagnosed as having Ramsay Hunt syndrome and treated with oral valacyclovir. However, the symptoms did not improve, and bilateral hearing loss and inspiratory stridor appeared. She was transferred to our hospital for further treatment. Subsequently, she developed not only bilateral sensorineural hearing loss, subglottic swelling, and scleritis, but also bilateral auricular swelling. She was finally diagnosed as having RP by Damiani’s criteria. Facial nerve palsy is not inconsistent with RP, although neurological symptoms in RP are rare. Oral prednisolone 40 mg/day improved the symptoms dramatically. She was misdiagnosed as having Ramsay Hunt syndrome because her unilateral facial nerve palsy and auricular swelling were on the same side, and the other auricle was not initially involved. There are few reports of RP with facial nerve palsy as the first symptom. Moreover, it is novel that, based on this report, it appears that RP and Ramsay Hunt syndrome need to be distinguished.

We need to consider RP when the patient presents with both facial nerve palsy and auricular swelling, even though facial nerve palsy in RP is rare.