Erythromelalgia: Vasculopathy, Neuropathy, or Both?

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Abstract
ERYTHROMELALGIA IS a clinical syndrome characterized by intermittent (though constant in some patients) heat, redness, and pain affecting the extremities (usually the lower extremities, and usually bilaterally and symmetrically). Local heat during episodes is such a key component of the syndrome that the term "erythermalgia" has been proposed to better describe it.1 Patients with erythromelalgia present not only to their internists or family physicians but also to specialists in dermatology, vascular medicine and surgery, cardiology, neurology, and pain management. The clinical characteristics of patients with erythromelalgia have been described.1-3 Although severity varies widely, the syndrome is associated with diminished quality-of-life scores, primarily because of the pain associated with episodes of erythromelalgia.2 Compared with an age- and sex-matched population, an increased mortality rate was also noted.2