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April 1966

Erythermalgia: A Clue to the Early Diagnosis of Myeloproliferative Disorders

Author Affiliations
From the Mayo Clinic and Mayo Foundation: Section of Medicine (Drs. Fairbairn and Hagedorn). Mayo Graduate School of Medicine, University of Minnesota (Drs. Alarcón-Segovia and Babb).
Arch Intern Med. 1966;117(4):511-515. doi:10.1001/archinte.1966.03870100039008

ERYTHERMALGIA is a disorder characterized by burning distress of the extremities accompanied by redness and increased temperature of the skin. These symptoms are often initiated by an increase in environmental temperature and relieved by measures that cool the skin.1 Smith and Allen 2 divided erythermalgia into primary and secondary forms depending on whether or not there were associated disease states in the neurologic, vascular, or hematologic systems. In a recent study of 51 cases of erythermalgia seen at the Mayo Clinic in the years 1951 through 1960 we found 21 cases of the secondary form.3 Of particular interest was our finding that in eight patients with a myeloproliferative disease the "secondary" erythermalgia preceded recognition of the hematologic disorder by up to 16 years. We report these cases herein to call attention to this early diagnostic clue.

Material  The myeloproliferative syndrome may be defined as an unexplained, persistent proliferation

It is recognized that criteria currently in use for the diagnosis of polycythemia vera are more rigid than those of earlier years as represented by our cases. Plasma volume was determined in six patients by the Congo red method. Recent use of more refined, radioisotope techniques in our laboratory indicates that the older method may be unreliable, and therefore we have discarded the early data.
The Scripps Clinic, La Jolla, Calif, supplied this follow-up information.
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