Erythermalgia. A clue to the early diagnosis of myeloproliferative disorders

Abstract

Erythermalgia (erythromelalgia) is characterized by burning distress of the extremities accompanied by redness and increased temperature of the skin. These symptoms are often initiated by increased environmental temperature and relieved by cooling of the skin. Erythermalgia may be present long before a hematologic abnormality is recognized, and thus it can be an early clue to myeloproliferative disorders. In 7 of 51 cases of erythermalgia this syndrome preceded for as long as 16 years the detection of poly-cythermia vera and in one it antedated recognition of agnogenic myeloid metaplasia by 7 years. These 8 cases are reported.