What causes secondary primary erythromelalgia?

Updated: Nov 04, 2020
  • Author: Antoine N Saliba, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Most reported cases of secondary erythromelalgia are due to myeloproliferative disorders with thrombocytosis (most commonly polycythemia vera or essential thrombocytosis). The actual prevalence of erythromelalgia in myeloproliferative disorders is uncertain. Erythromelalgia manifests before the appearance of a myeloproliferative disorder in 85% of cases.

In the Mayo Clinic series, only 9% of erythromelalgia cases were associated with myeloproliferative disorders. [17] In the Norwegian series, 19% of cases were due to myeloproliferative disorders. [18]

Medications implicated in erythromelalgia include the following:

  • Bromocriptine [19]
  • The calcium channel blockers nifedipine, felodipine, and nicardipine
  • Topical isopropanol
  • Simvastatin
  • Pergolide (withdrawn from the US market in 2007)

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