Abstract
Erythromelalgia is a term that describes the clinical syndrome of red, hot extremities. This rare disorder has been described and characterized in patients seen at the Mayo Clinic in Rochester, Minn, for many decades.1Davis MD O'Fallon WM Rogers III, RS Rooke TW Natural history of erythromelalgia: presentation and outcome in 168 patients.Arch Dermatol. 2000; 136: 330-336Crossref PubMed Google Scholar The lower extremities are involved more frequently than are the upper extremities, and involvement is often symmetrical. Symptoms can be intermittent or, more rarely, constant. The pain can be severe and debilitating; patients attempt to cool the affected areas. Primary and secondary forms of erythromelalgia have been described. Polycythemia vera and other myeloproliferative disorders may underlie the secondary form.2Tefferi A Polycythemia vera: a comprehensive review and clinical recommendations.Mayo Clin Proc. 2003; 78: 174-194Abstract Full Text Full Text PDF PubMed Scopus (126) Google Scholar Recent evidence indicates that a small-fiber neuropathy is associated with erythromelalgia.3Davis MD Sandroni P Rooke TW Low PA Erythromelalgia: vasculopathy, neuropathy, or both? a prospective study of vascular and neurophysiologic studies in erythromelalgia.Arch Dermatol. 2003; 139: 1337-1343Crossref PubMed Scopus (109) Google Scholar Treatment is difficult; aspirin, nonsteroidal anti-inflammatory agents, anticonvulsants including gabapentin, tricyclic antidepressants, and vasoactive drugs have all been tried. Response varies. Over time, the symptoms can worsen, stay the same, improve, or resolve in approximately equal proportions.
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