[Skip to Content]
[Skip to Content Landing]
Article
April 1966

Erythermalgia: A Clue to the Early Diagnosis of Myeloproliferative Disorders

Author Affiliations
ROCHESTER, MINN
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
Abstract

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

References
1.
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.
2.
The Scripps Clinic, La Jolla, Calif, supplied this follow-up information.
3.
Allen, E.V.; Barker, N.W.; and Hines, E.A., Jr.:  Peripheral Vascular Diseases , ed 3, Philadelphia: W. B. Saunders Company, 1962, pp 1044.
4.
Smith, L.A., and Allen, E.V.:  Erythermalgia (Erythromelalgia) of Extremities: Syndrome Characterized by Redness, Heat, and Pain ,  Amer Heart J 16:175-188 ( (Aug) ) 1938.Crossref
5.
Babb, R.R.; Alarcón-Segovia, D.; and Fair-bairn, J.F., II:  Erythermalgia: Review of 51 Cases ,  Circulation 29:136-141 ( (Jan) ) 1964.Crossref
6.
Dameshek, W., and Gunz, F.:  Leukemia , ed 2, New York: Grune & Stratton, Inc., 1964, pp 356402.
7.
Wasserman, L.R.:  Polycythemia Vera—Its Course and Treatment: Relation to Myeloid Metaplasia and Leukemia ,  Bull NY Acad Med 30:343-375 ( (May) ) 1954.
8.
Brown, G.E., and Griffin, H.Z.:  Studies of the Vascular Changes in Cases of Polycythemia Vera ,  Amer J Med Sci 171:157-168 ( (Feb) ) 1926.Crossref
9.
Nakai, G.S.; Craddock, C.G.; and Figueroa, W.G.:  Agnogenic Myeloid Metaplasia: A Survey of 29 Cases and a Review of the Literature ,  Ann Intern Med 57:419-440 ( (Sept) ) 1962.Crossref
10.
Osler, W.:  Chronic Cyanosis With Polycythemia and Enlarged Spleen: A New Clinical Entity ,  Amer J Med Sci 126:187-201, 1903.Crossref
11.
Osler, W., Jr.:  A Clinical Lecture on Erythraemia: (Polycythaemia With Cyanosis, maladie de Vaquez) ,  Lancet 1:143-146 ( (Jan 18) ) 1908.
12.
Harrop, G.A., Jr.:  Polycythemia ,  Medicine 7:291-344 ( (Aug) ) 1928.
13.
Norman, I.L., and Allen, E.V.:  Vascular Complications of Polycythemia ,  Amer Heart J 13:257-274 ( (March) ) 1937.Crossref
14.
Oppenheimer, B.S.:  Vascular Occlusion in Polycythemia Vera ,  Trans Acad Amer Physicians 44:338-344, 1929.
15.
Burris, M.B., and Arrowsmith, W.R.:  Vascular Complications of Polycythemia Vera: A Study of 68 Cases ,  Surg Clin N Amer ( (Aug) ), 1953, pp 1023-1028.
16.
Calabresi, P., and Meyer, O.O.:  Polycythemia Vera: I. Clinical and Laboratory Manifestations ,  Ann Intern Med 50:1182-1202 ( (May) ) 1959.Crossref
17.
Lawrence, J.H.; Berlin, N.I.; and Huff, R.L.:  The Nature and Treatment of Polycythemia: Studies of 263 Patients ,  Medicine 32:323-388 ( (Sept) ) 1953.Crossref
18.
Brown, G.E., and Giffin, H.Z.:  Peripheral Arterial Disease in Polycythemia Vera ,  Arch Intern Med 46:705-717 ( (Oct) ) 1930.Crossref
19.
Dameshek, W., and Henstell, H.H.:  The Diagnosis of Polycythemia ,  Ann Intern Med 13(pt 2): 1360-1387 ( (Feb) ) 1940.Crossref
20.
Lewis, T.:  Clinical Observations and Experiments Relating to Burning Pain in the Extremities, and to So-Called "Erythromelalgia" in Particular ,  Clin Sci 1:175-211 ( (Dec 21) ) 1933.
21.
Lewis, T., and Hess, W.:  Pain Derived From the Skin and the Mechanism of Its Production ,  Clin Sci 1:39-61 ( (July 21) ) 1933.
×