Erythromelalgia of the Ears: An Unusual Variant and Response to Therapy

Erythromelalgia is characterized by episodes of erythematous, warm, burning acral skin, which is exacerbated by heat and relieved by cold. Erythromelalgia usually affects the feet and/or hands but, although rare, erythromelalgia may affect the ears. The authors present a 65-year-old woman with erythromelalgia of the ears with disabling symptoms whose diagnosis was delayed for 6 years. The patient failed to respond to numerous therapies before rapidly improving with oral amitriptyline and amitriptyline 1% to 2% and ketamine 0.5% to 1% topical gel.