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Erythromelalgia

Author
Mark DP Davis, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Erythromelalgia is a rare, acquired or (very rarely) inherited clinical syndrome of intermittently red, hot, painful extremities. The syndrome usually affects the lower extremities (predominantly the feet) but may also involve the upper extremities (predominantly hands) and rarely concomitantly involves the face. Patients instinctively try to relieve symptoms by cooling the involved areas with fans, cold water, or ice.

There is no cure for erythromelalgia; therefore, treatment is focused on improving symptoms. Many patients can be successfully managed with behavioral interventions, topical medications, and aspirin. Other interventions may be beneficial when these measures are insufficient.

The epidemiology, clinical features, diagnosis, and management of erythromelalgia will be reviewed here.

EPIDEMIOLOGY

Epidemiologic data on erythromelalgia are limited, but erythromelalgia appears to be rare [1-4]. Population-based studies from the United States, Sweden, and Norway have found incidence rates of less than 2 per 100,000 people per year [1-3].

Erythromelalgia is more common in women than in men and most often occurs in adults [1,2]. In a population-based study in Olmsted County, Minnesota, the age-adjusted incidence rates were 2 (95% CI 1.2-2.7) per 100,000 women and 0.6 (95% CI 0.1-1.1) per 100,000 men [1]. The median age at diagnosis was 61 years (range, 16 to 90 years). Among 27 patients with erythromelalgia identified in a Swedish study, the median age was 49 years (interquartile range 34 to 68 years) [2].

                     

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Literature review current through: May 2017. | This topic last updated: May 31, 2017.
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