eMedicine Specialties > Hematology > Stem Cells and Disorders

Erythromelalgia: Treatment & Medication

Author: Robert J Nardino, MD, Director, Internal Medicine Residency Program, Assistant Clinical Professor, Department of Internal Medicine, Hospital of Saint Raphael, Yale University School of Medicine
Coauthor(s): Andrea LM Silber, MD, Associate Clinical Professor of Medicine, Section of Hematology-Oncology, Yale University School of Medicine
Contributor Information and Disclosures

Updated: Aug 13, 2009

Treatment

Medical Care

  • Evaluation and treatment can be conducted in an outpatient setting.
  • Local measures, such as cooling or elevating the extremity, may relieve symptoms.
  • In patients with myeloproliferative disorders, chemotherapy to reduce the platelet count often alleviates symptoms, but it is not universally effective.
  • Some patients with polycythemia vera have responded to phlebotomy.

Surgical Care

  • Surgery probably has no role except to treat the rare complication of gangrene.
  • Surgical sympathectomy has been attempted, with variable results.

Consultations

Hematologist, if an associated myeloproliferative disorder is present

Diet

No specific dietary restrictions are necessary.

Activity

Exercise may induce an acute episode.

Medication

Platelet inhibition is helpful in patients with thrombocytosis. Aspirin is most often used, but anagrelide may also be effective.5

The optimal pharmacologic therapy in primary erythromelalgia is still unknown. Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, gabapentin, and typhoid vaccine appear in the literature, as well as case series with high-dose magnesium, intravenous bupivacaine, the lidocaine patch, and lidocaine plus mexiletine. The present author knows of no randomized trial of therapy for erythromelalgia. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analog) improved symptoms.

Nonsteroidal anti-inflammatory agents

Aspirin is preferred because it provides relief lasting longer than that of indomethacin or other nonsteroidal anti-inflammatory drugs (NSAIDs), presumably because of irreversible platelet inhibition.


Aspirin (Anacin, Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin)

Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.

Adult

500 mg PO; a single dose often relieves symptoms; maximum recommended dose 4 g/d (650 mg PO q4h)

Pediatric

10-15 mg/kg PO

Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses > 2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs

Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma; due to association of aspirin with Reye syndrome, do not use in children ( <16 y) with flu

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in severe anemia, in history of blood coagulation defects, and in patients taking anticoagulants

More on Erythromelalgia

Overview: Erythromelalgia
Differential Diagnoses & Workup: Erythromelalgia
Treatment & Medication: Erythromelalgia
Follow-up: Erythromelalgia
Multimedia: Erythromelalgia
References
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References

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  2. Michiels JJ, Drenth JP, Van Genderen PJ. Classification and diagnosis of erythromelalgia and erythermalgia. Int J Dermatol. Feb 1995;34(2):97-100. [Medline].

  3. Kalgaard OM, Seem E, Kvernebo K. Erythromelalgia: a clinical study of 87 cases. J Intern Med. Sep 1997;242(3):191-7. [Medline].

  4. Davis MD, O'Fallon WM, Rogers RS 3rd, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol. Mar 2000;136(3):330-6. [Medline].

  5. Cacciola RR, Cipolla A, Di Francesco E, et al. Treatment of symptomatic patients with essential thrombocythemia: effectiveness of anagrelide. Am J Hematol. Sep 2005;80(1):81-3.

  6. Alarcon-Segovia D, Babb RR, Fairbairn JF, Hagedorn AB. Erythermalgia. A clue to the early diagnosis of myeloproliferative disorders. Arch Intern Med. Apr 1966;117(4):511-5. [Medline].

  7. Charkoudian N. Skin blood flow in adult human thermoregulation: how it works, when it does not, and why. Mayo Clin Proc. May 2003;78(5):603-12. [Medline].

  8. Cohen JS. High-dose oral magnesium treatment of chronic, intractable erythromelalgia. Ann Pharmacother. Feb 2002;36(2):255-60. [Medline].

  9. Davis MD, Sandroni P. Lidocaine patch for pain of erythromelalgia: follow-up of 34 patients. Arch Dermatol. Oct 2005;141(10):1320-1.

  10. Davis MD, Sandroni P, Rooke TW, Low PA. Erythromelalgia: vasculopathy, neuropathy, or both? A prospective study of vascular and neurophysiologic studies in erythromelalgia. Arch Dermatol. Oct 2003;139(10):1337-43. [Medline].

  11. Drenth JP, te Morsche RH, Guillet G, et al. SCN9A mutations define primary erythermalgia as a neuropathic disorder of voltage gated sodium channels. J Invest Dermatol. Jun 2005;124(6):1333-8.

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  26. Colon Y, Tokarz KA. Patient education and self-advocacy: queries and responses on pain management; erythromelalgia. J Pain Palliat Care Pharmacother. Sep 2009;23(3):295-7. [Medline].

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Further Reading

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Keywords

erythromelalgia, erythermalgia, primary erythromelalgia, secondary erythromelalgia, myeloproliferative disorder, arteriolar fibrosis, idiopathic erythromelalgia, platelet-mediated erythromelalgia, aspirin-sensitive erythromelalgia, primary erythermalgia, secondary erythermalgia, polycythemia vera, essential thrombocytosis, peripheral vascular disease

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Contributor Information and Disclosures

Author

Robert J Nardino, MD, Director, Internal Medicine Residency Program, Assistant Clinical Professor, Department of Internal Medicine, Hospital of Saint Raphael, Yale University School of Medicine
Robert J Nardino, MD is a member of the following medical societies: American College of Physicians, Association of Program Directors in Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Andrea LM Silber, MD, Associate Clinical Professor of Medicine, Section of Hematology-Oncology, Yale University School of Medicine
Andrea LM Silber, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Society of Preventive Oncology
Disclosure: sanofi Aventis Honoraria Speaking and teaching; novartis Honoraria Speaking and teaching; gsk Honoraria Speaking and teaching

Medical Editor

Rodger L Bick, MD, PhD, FACP, Clinical Professor of Medicine, University of Texas Southwestern Medical Center; Director, Dallas and Pacific Thrombosis Hemostasis and Vascular Medicine Clinical Center
Rodger L Bick, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Blood Banks, American Cancer Society, American College of Angiology, American College of Physicians, American Geriatrics Society, American Heart Association, American Medical Association, American Society for Clinical Pathology, American Society of Hematology, Association of Clinical Scientists, California Medical Association, California Thoracic Society, International College of Angiology, International Society of Hematology, International Society on Thrombosis and Haemostasis, New York Academy of Sciences, and Southwest Oncology Group
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Troy H Guthrie, Jr, MD, Director of Cancer Institute, Baptist Medical Center
Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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