Erythromelalgia

Erythromelalgia is a rare syndrome characterised by attacks of severe burning pain, erythema, and warmth of the extremities-primarily the feet and, to a lesser extend, the hands. The distress is induced by environmental heat, exercise, and dependency; it is relieved by exposure to cold and elevation of the extremity. Idiopathic and secondary forms of erythromelalgia exist. Secondary erythromelalgia has been linked to a wide variety of diseases, the most common of which are certain myeloproliferative disorders. This type affect adults only, and is dramatically ameliorated by small doses of aspirin. Intravascular platelet activation and aggregation, with plugging of arterioles have been suggested as the cause of erythromelalgia in myeloproliferative disorders. The pathogenesis of idiopathic form has not yet been delineated.