Erythromelalgia is a paroxysmal bilateral vasodilatory disorder of unknown cause. Idiopathic (primary) erythromelalgia occurs in otherwise healthy persons and affects men and women equally. A secondary type is occasionally seen in patients with polycythemia vera, hypertension, gout, and neurologic diseases.
The chief symptoms are erythema, warmth, and bilateral burning pain that lasts minutes to hours, at first involving circumscribed areas on the balls of the feet or palms and often progressing to involve the entire extremity. Symptoms occur in response to vasodilation produced by exercise or heat and can be prominent at night when the extremities are warmed under bedclothes. Relief may be obtained by cooling and elevating the affected extremity.
No findings are generally present between attacks. With onset of an attack, skin temperature and arterial pulsations are increased and the involved areas are warm, erythematous, and sweaty.
In primary erythromelalgia, aspirin (650 mg every 4-6 hours) often provides excellent relief and may in fact be diagnostic. Warm environments are to be avoided. ß-Blockers, epidural corticosteroid injections, and lidocaine patches have reported anecdotal success. Secondary erythromelalgia may improve with treatment of the primary disease process.
Davis MD et al: Lidocaine patch for pain of erythromelalgia. Arch Dermatol 2002;138:17.
Stricker LJ et al: Resolution of refractory symptoms of secondary erythermalgia with intermittent epidural bupivacaine. Reg Anesth Pain Med 2001;26:488.
Revision date: June 18, 2011
Last revised: by Jorge P. Ribeiro, MD