The syndrome of erythermalgia is characterized by a burning distress of the extremities that is accompanied by redness and increased temperature of the skin. These symptoms are initiated or exacerbated by an increase in environmental temperature and diminished by measures that cool the skin. Of 51 patients with this clinical syndrome seen at the Mayo Clinic during the years 1951 to 1960 inclusive, 30 were considered as having primary erythermalgia because of the absence of demonstrable associated conditions, and the rest were classified as having secondary erythermalgia because the condition was associated with various diseases. Particularly significant was the relation of erythermalgia to the myeloproliferative disorders as evidenced in 10 cases. In some of these cases, erythermalgia preceded other manifestations of the myeloproliferative disorder by as long as 12 years. The primary type was found to occur in younger individuals and to be more often bilateral, to produce pain of greater intensity, and to involve larger areas of the affected extremities. The pathologic physiology of this syndrome remains unknown.
Babb et al. (Wed,) studied this question.