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Genetic and Rare Diseases Information Center (GARD)

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Erythromelalgia


Other Names for this Disease

  • Mitchell disease (formerly)
  • Primary erythermalgia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I suffer from erythromelalgia. I recently began to take gabapentin without much relief. Can this medication successfully treat erythromelalgia? What other treatment options are available?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is erythromelalgia?

Erythromelalgia (EM) is a rare condition characterized by episodes of burning pain, warmth, swelling and redness in parts of the body, particularly the hands and feet. This condition may occur spontaneously (primary EM) or secondary to neurological diseases, autoimmune diseases, or myeloproliferative disorders (secondary EM). Episodes may be triggered by increased body temperature, alcohol, and eating spicy foods. About 15% of cases are caused by mutations in the SCN9A gene and are inherited in an autosomal dominant manner. Other cases may be caused by unidentified genes or by non-genetic factors. Treatment depends on the underlying cause and may include topical and/or oral medications. In some cases, the condition goes away without treatment.[1][2]
Last updated: 12/11/2013

What treatment is available for erythromelalgia?

There appear to be several subtypes of erythromelalgia and different subtypes respond to different therapies. Treatment consists of a trying various approaches until the best therapy is found.[3] Patients respond quite variably to drug therapy and no single therapy has proved consistently effective. Spontaneous remissions have also been known to occur.[4]

Drugs shown to be effective in relieving pain in some individuals include: aspirin, prostaglandins (misoprostol), serotonin-norepinephrine reuptake inhibitors (venlafaxine and sertraline) and selective serotonin reuptake inhibitors (SSRIs), anticonvulsants (gabapentin), sodium channel blockers, carbamazepine, tricyclic antidepressants (amitriptyline and imipramine), calcium antagonists (nifedipine and diltiazem), magnesium, sodium nitroprusside infusion, and cyclosporine. Other treatments include: cooling or elevating the extremity, topical treatment with capsaicin cream, and surgical sympathectomy (a procedure where the sympathetic nerve fibers are selectively cut).Avoidance of triggers (such as warmth, prolonged standing, etc.) may reduce the number or severity of flare ups. [3][4]

Last updated: 3/27/2014

Has gabapentin been used to successfully treat erythromelalgia?

While we do not provide medical advice, the literature states that gabapentin has been successfully used as a treatment for erythromelalgia.[5][6][7] Gabapentin is an anticonvulsant that treats seizures by decreasing abnormal excitement in the brain. It relieves pain by changing the way the body senses pain.[8]
Last updated: 4/1/2009

Where can I find more information about using gabapentin as a treatment for erythromelalgia?

PubMed lists journal articles that discuss the treatment of erythromelalgia with gabapentin. Click on the link to go to PubMed and review citations to these articles.
Last updated: 4/1/2009

What new therapy for erythromelalgia may be of interest?

In an article titled “Erythromelalgia: New theories and therapies,” Dr. Jay S. Cohen discusses a wide variety of possible approaches including the use of topical treatments, oral medications, drug infusions, surgical intervention, medical devices and non-medical therapies.  
Last updated: 4/1/2009

How can I locate additional medical articles about treatment for erythromelalgia?

You can find relevant journal articles on treatment for erythromelalgia through a service called PubMed, a searchable database of medical literature. Information on finding an article and its title, authors, and publishing details is listed here. Some articles are available as a complete document, while information on other studies is available as a summary abstract. To obtain the full article, contact a medical/university library (or your local library for interlibrary loan), or order it online using the following link. Using "treatment AND erythromelalgia" as your search term should locate articles. To narrow your search, click on the “Limits” tab under the search box and specify your criteria for locating more relevant articles.  Click here to view a search.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed

The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.
Last updated: 6/23/2011

References
Other Names for this Disease
  • Mitchell disease (formerly)
  • Primary erythermalgia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.