ERYTHROMELALGIA

By Gary M. White, MD


Erythromelalgia is a rare condition characterized by red, hot, painful extremities.

Clinical

The palms and soles are chronically red with paroxysmal burning pain and increased skin temperature. The feet are more commonly affected and flares are precipitated by dependency, heat and exercise. Swelling of the feet may occasionally be seen. Attacks may last minutes to hours, and in severe cases, the symptoms may be continuous. Relief is achieved by cooling the affected area. Patients have been known to sleep with their feet outside the covers or through windows. Some have walked barefoot in the snow or stuck their feet in a refrigerator to obtain relief.

Diagnosis

Five key diagnostic criteria have been proposed [Clin Orthop. 1979;144;249]:

The diagnostician often has to rely on a good medical history as the clinical findings are often not present in the office.

Treatment

Patients often find relief by soaking their hands or feet in cold water or by elevation. Aspirin 500 mg/day to inhibit platelet aggregation can be dramatically helpful, especially when there is thrombocytosis. Venlafaxine 37.5 mg BID, a serotonin reuptake inhibitor, was helpful in two of three patients. Topically applied midodrine, 0.2%, an α1-Agonist, has been used [JAMA Derm 2015;151;1025]. Oral cetirizine hydrochloride (2.5mg/kg/once daily) partially helped one child [J Med Case Rep. 2014 Feb 25;8:69].

References

Ear erythromelalgia. A 7-year-old boy presented with a 2-year history of burning sensation and pain of his external ears. There was a history of an intermittent sudden onset of burning sensation and erythema of both of his ears, lasting about 20 minutes. The patient reported experiencing approximately 7 of these episodes per day, which required immediate ice pack application to the ears to attain symptomatic relief. Pediatric-Isolated Auricular Erythromelalgia: A Case Report Case Rep Pediatr. 2012; 2012: 854081

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