ADULTS ERYTHROMELALGIA - ABOUT 16 CASES

The authors report sixteen consecutive cases of erythromelalgia, an infrequent disease in which local heat, redness, and pain develop in the hands and/or feet in recurrent attacks. The disease was essential in nine patients ; in the remaining seven, the cause was a myeloproliferative syndrome (polycythemia vera in 3 cases and thrombocythemia in 1 case) or a drug (bromocriptine, nicardipine, and nifedipine, one case each). Acetylsalicylic acid was effective in only six of the nine essential cases. Intravascular platelet activation and aggregation with plugging of the arterioles has been suggested as the mechanism of erythromelalgia in patients with myeloproliferative disorders. Other, as yet unelucidated pathophysiologic events underlie the juvenile-onset forms, which usually fail to respond to acetylsalicylic acid.