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Warmth, congestion, redness, and burning pain in a limb when it is exposed to heat
    Differential Diagnosis[Top] [Nav]
erythromelalgia
    Discussion -- not available online[Top] [Nav]
    Erratum in printed version[Top] [Nav]
       *[footnote 3] Title of the book printed incorrectly.
    Update Comments[Top] [Nav]
  • Distinguishes 3 types of erythromelalgia:
    1. associated with thrombocythemia -- a consequence of platelet-mediated arteriolar inflammation and thrombosis. Frequently asymmetric, relief of pain for several days after a single dose of aspirin confirms the diagnosis.
    2. primary erythermalgia -- a rare disorder beginning in childhood or adolescence. Refers to strict diagnostic criteria, and found only 13 cases published.
    3. secondary erythermalgia -- associated with gout, cryoglobulinemia, systemic lupus erythematosis, diabetes, vasoactive drugs, and more. Platelets seem not to play a role here, aspirin has no effect, and relief is best obtained by treating the underlying disorder.
    [101]
  • Has a few paragraphs about thrombocythemia, but blunders by discussing "erythromyalgia." [102]
  • Hints that erythromelalgia can be mistaken for Raynaud phenomenon, which is odd, given the exactly opposite effects of cold water immersion noted above (McKusick). [103]
    Footnotes in Print Edition[Top] [Nav]
    (1) Erythermalgia: review of 51 cases.
  Babb RR, et al.   Circulation. 1964;29:136-141.  Medline
    (2) Fabry disease: impaired autonomic function.
  Cable WJ, Kolodny EH, et al.   Neurology 1982 May;32(5):498-502.  Pubmed+Abstract  Similars
    (3) The Genuine Works of Hippocrates.
  Adams F (transl). Huntington, NY: Robert E. Krieger, 1972: Book of Prognostics, paragraph 3.
    (4) Erythromelalgia and myeloproliferative disorders.
  Kurzrock R, Cohen PR.   Arch Intern Med 1989 Jan;149(1):105-9.  Pubmed+Abstract  Similars
    (5a) Erythromelalgia caused by platelet-mediated arteriolar inflammation and thrombosis in thrombocythemia.
  Michiels JJ, Abels J, et al.   Ann Intern Med 1985 Apr;102(4):466-71.  Pubmed+Abstract  Similars
Notes that intensity of the pain correlates well with the temperature of the skin.
    (5b) Erythromelalgia without thrombocythemia [letter].
  Alarcon-Segovia D.   Ann Intern Med 1985 Sep;103(3):473.  Pubmed  Similars
    (M) McKusick 133020 = familial primary erythermalgia.
Notes, interestingly, that erythromelalgia is the antithesis of Raynaud syndrome, in that immersion of the afflicted limb in ice water relieves discomfort. Also shows that pain of Fabry disease can be confused with erythromelalgia.
    New References[Top] [Nav]
    101.Three types of erythromelalgia [editorial] [see comments].
  Drenth JP, Michiels JJ.   BMJ 1990 Sep 8;301(6750):454-5.  Pubmed  Similars
    102.Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 17-1992. Repeated bouts of hematochezia in an 80-year-old hypertensive man [published erratum appears in N Engl J Med 1992 Nov 26;327(22):1612].
  N Engl J Med 1992 Apr 23;326(17):1137-46.  Pubmed  Similars
    103.Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 35-1988. 63-year-old asplenic man with myelofibrosis, fever, and bloody diarrhea.
  N Engl J Med 1988 Sep 1;319(9):564-74.  Pubmed  Similars
    104.Classification and diagnosis of erythromelalgia and erythermalgia.
  Michiels JJ, Drenth JP, et al.   Int J Dermatol 1995 Feb;34(2):97-100.  Pubmed  Similars
    105.Paraneoplastic erythromelalgia.
  Kurzrock R, Cohen PR.   Clin Dermatol 1993 Jan-Mar;11(1):73-82.  Pubmed  Similars
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    ©1986-2000 John Sotos, MD. All rights reserved.  Last updated 16:35 PDT on July 4, 2000.[Top]

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