Erythromelalgia (EM)

What is erythromelalgia (EM)?

Erythromelalgia (EM) is a rare condition that typically affects the extremities. It causes burning, warmth, redness, and swelling. These symptoms can occur periodically or continuously. It usually affects both sides of the body, but can affect only one side as well. EM’s course and severity varies from person to person. The onset can be gradual with only mild symptoms, or it can come on suddenly, spread, and become severe in a short period of time. 
EM may occur on its own (primary EM) or develop secondary to another neurological, autoimmune, or myeloproliferative disorder (secondary EM). In some cases, the disease may go away on its own without treatment, but in other cases treatment is required. Symptoms of EM may be triggered by things like alcohol, eating spicy foods, exercising, and other causes of increased body temperature. The burning and pain experienced can be extreme. For some people, wearing tight shoes, socks, or gloves can trigger a very painful flare up. Mild signs and symptoms may develop in childhood and worsen/spread with age. 
EM is sometimes characterized as a peripheral neuropathy because it affects the peripheral nervous system (PNS). The peripheral nervous system connects the brain and spinal cord to muscles and cells that detect sensations. 
Risk factors
  • Gender: EM is more common in females
  • Age: Onset is most common in middle age, but EM can occur at any age. 
  • Genetics
  • Having a neurological, autoimmune, or myeloproliferative disorder
In order to diagnose EM, your doctor will likely do a complete medical history and physical exam. He/she will pay close attention to your symptoms and testing will be done to rule out other conditions. 
There is no specific test that can be done to diagnose EM. The tests that are done are typically used to rule out or look for other possible conditions. 
Some tests that may be done to diagnose EM include:
  • Imaging studies such as X-rays of the hands and feet may be done. In people with EM, imaging studies will likely show no significant findings. 
  • Blood tests may be done to look for signs/evidence of a myeloproliferative disorder 
  • Genetic testing may be done in people with primary erythromelalgia to look for a mutation in the SCN9A gene - which can be common in people with primary erythromelalgia. 
  • Thermography testing may be done to test for increased skin temperatures in affected areas. 
Treatment type and success may vary from person to person. The aim of treatment is symptom relief. In some cases, the condition may even go into remission without treatment. Avoiding things that increase one’s body temperature may be effective in preventing painful flare ups. 
Potential treatment options for erythomelalgia include:
  • Treatment of any underlying diseases/conditions 
  • Aspirin
  • Serotonin norepinephrine reuptake inhibitors
  • Selective serotonin reuptake inhibitors 
  • Sodium channel blockers
  • Anticonvulsant medications
  • Pain medications
  • Tricyclic antidepressants
  • Calcium antagonists
  • Cooling/elevating extremities
  • Topical ointment
  • Epidural opiate infusions
  • Surgical sympathectomy 
  • Magnesium
  • Early onset erythromelalgia is rare
  • The prevalence of erythromelalgia in people with myeloproliferative disorders is 3-65%
  • 1.3 out of every 100,000 people in the U.S. are diagnosed with EM each year
  • 2.0 women out of every 100,000 people in the U.S. are diagnosed with EM each year
  • The average age of diagnosis is 61
about erythromelalgia (EM)

  • "Erythromelalgia.” WebMD. Medscape, 31 Jul. 2017. Web. 29 Sep. 2017. 
  • “What is EM?” The Erythromelalgia Association. TEA, n.d. Web. 29 Sep. 2017. 
  • “Erythromelalgia.” National Organization for Rare Disorders. NORD, n.d. Web. 29 Sep. 2017. 
  • “Erythromelalgia.” National Institutes of Health National Center for Advancing Translational Sciences. Genetic and Rare Diseases Information Center, n.d. Web. 29 Sep. 2017. 
  • “Erythromelalgia.” WebMD, 19 Jan. 2012. Web. 29 Sep. 2017. 
  • “erythromelalgia.” National Institutes of Health U.S. National Library of Medicine. Genetics Home Reference, 26 Sep. 2017. Web. 29 Sep. 2017. 
  • “Erythromelalgia.” Merck Sharp & Dohme Corp. Merck & Co, n.d. Web. 29 Sep. 2017.  

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