What is erythromelalgia?

Updated: Sep 17, 2018
  • Author: Antoine N Saliba, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness of the extremities. [1, 2] Despite the controversy regarding nomenclature and classification, a distinction is generally made between primary (idiopathic or genetic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders), as well as between early- and late-onset disease.

The name of this disorder is derived from three Greek words: erythros (“red”), melos (“limb”), and algos (“pain”). Mitchell first described erythromelalgia in the 1870s. Smith and Allen further categorized it in 1938, proposing the term erythermalgia to emphasize the characteristic warmth. They also suggested a distinction between primary (idiopathic) erythromelalgia and secondary erythromelalgia (due to underlying neurologic, hematologic, or vascular problems).

Drenth and Michiels distinguished between erythromelalgia and erythermalgia on the basis of responsiveness to aspirin and established the following three categories [3] 3:

  • Erythromelalgia (platelet mediated and aspirin sensitive)
  • Primary erythermalgia
  • Secondary erythermalgia

Investigation into the pathophysiology of primary erythromelalgia indicates that this disorder may serve as a model for understanding common chronic pain conditions.

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