What is erythromelalgia?

According to the National Organization for Rare Disorders, erythromelalgia is a rare condition that is estimated to affect only 2 out of every 100,000 people. Its three primary symptoms – warmth, red skin (erythema), and burning pain – are usually felt in the legs and arms, particularly in the hands and feet. If erythromelalgia is affecting your feet, you may notice the soles of your feet burning, tingling, and looking bright red.

What is erythromelalgia?

The condition is chronic, but most individuals experience flare-ups rather than continuous symptoms. It is considered a peripheral neuropathy, affecting the nerves that branch out from the central nervous system.

What causes erythromelalgia?

Unfortunately, the precise causes of erythromelalgia are hard to pinpoint, as the condition is still not well understood, but abnormalities in the widening and narrowing of the blood vessels are believed to play a part. Changes in the nerves responsible for sweating can also play a role.

There may also be a genetic component of erythromelalgia; about 5% of erythromelalgia patients have a family history of the condition.

Primary erythromelalgia

Primary erythromelalgia occurs on its own and not as the result of any health condition or medical concern. This type of primary disease (also called “idiopathic” erythromelalgia) can have a sudden onset, commonly appearing when individuals are in their 20s.

Inherited erythromelalgia — erythromelalgia caused by a gene mutation — is also considered primary.

Secondary erythromelalgia

Secondary erythromelalgia is the result of another underlying disease or disorder. Medical conditions that can result in secondary erythromelalgia include:

  • Neuropathy — damaged or defective nerves
  • Myeloproliferative disorders — diseases of the blood and bone marrow
  • Autoimmune diseases — including multiple sclerosis, lupus, and rheumatoid arthritis
  • Essential thrombocythemia — a blood disorder that causes the production of too many platelets
  • Raynaud’s phenomenon — a disorder that causes restricted blood flow

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Should I be worried about the symptoms of erythromelalgia?

Erythromelalgia presents three common symptoms: burning sensations, redness, and warmth. Erythromelalgia usually flares up on both sides of the body, but can sometimes occur asymmetrically.

Other symptoms can include:

  • Swelling
  • Sweating of the affected areas
  • Skin that is tender to the touch

Any of these symptoms can range from mild to severe. Mild symptoms generally don’t affect quality of life and can often be managed. But very swollen and painful skin can cause significant disruption, making it hard to go about your daily routine, or even sleep comfortably.

Severe erythromelalgia of the feet can make walking difficult. As a result, it can affect your work and social life, and restrict your ability to be physically active in general.

What triggers erythromelalgia flare-ups?

While the exact cause of erythromelalgia isn’t known, increases in body temperature are thought to cause flare-ups. For example, if you experience erythromelalgia pain and burning in your feet, you may notice that wearing tight shoes and warm socks seems to set off symptoms.

Flare-ups can come on suddenly or gradually and last from a few minutes to a few days.

How is erythromelalgia diagnosed?

Because there are still no tests to definitively diagnose erythromelalgia, University Foot & Ankle Institute’s foot doctors will review your medical history and symptoms before performing a physical exam.

Because burning feet, pain, and redness can be signs of multiple problems, we will perform tests to rule out other potential problems. Testing may include X-rays, skin biopsies, or blood tests.

Erythromelalgia treatments and therapies

Depending on the suspected cause of erythromelalgia, various treatment options may be suggested. If you have secondary erythromelalgia, the focus should be on treating the underlying condition, not just managing symptoms. Most therapies aim to reduce flare-ups and manage symptoms.

Treatment options can include:

  • Trigger avoidance. Once you know what sets off a flare-up, taking steps to reduce those triggers may help decrease flare-up frequency. This can include not wearing tight shoes, controlling the temperature in your home, avoiding trigger foods (spicy foods, caffeine, alcohol, etc.), and dressing for the weather.
  • Cooling remedies. Cooling affected areas with fans or getting into an air-conditioned space can reduce pain during a flare-up. Avoid submerging the affected extremity in cold water or applying ice, as this can cause more damage.
  • Topical treatments. Lidocaine and capsaicin creams and gels may help soothe red and burning skin.
  • Oral medications. Several different oral medications have been found to reduce the frequency and severity of erythromelalgia symptoms. Working with the rest of your healthcare team, our doctors have a wide range of potential medications that can help with symptoms. Some examples include anticonvulsants, antidepressants, blood pressure medications, and beta blockers.
  • Infusions and injections. Intravenous medications for erythromelalgia are often reserved for severe flare-ups that do not respond to topical or oral medications.

Living with erythromelalgia

There isn’t a cure yet for erythromelalgia, which is often a chronic, lifelong condition. However, avoiding triggers can help reduce the frequency and severity of flare-ups.

Joining support groups like the Erythromelalgia Association, seeking guidance from our doctors, and taking steps to improve your overall health can also help improve your quality of life with erythromelalgia. 

Why trust UFAI with your feet?

The foot specialists, podiatrists, and surgeons at the University Foot and Ankle Institute have vast experience with all manner of foot problems, from erythromelalgia to fractures and foot ulcers. In addition, we have experts with custom orthotics, braces, and minimally invasive foot and ankle surgeries.

For a consultation, please call (877) 736-6001 or make an appointment online now.

University Foot and Ankle Institute is conveniently located throughout Southern California and the Los Angeles area. Our foot and ankle surgeons are available at locations in or near Santa Monica, Beverly Hills, West Los Angeles, Sherman Oaks, the San Fernando Valley, El Segundo, the South Bay, LAX, Calabasas, Agoura Hills, Westlake Village, Valencia, Santa Clarita, and Santa Barbara.

Sources:

Erythromelalgia – symptoms, causes, treatment: Nord. National Organization for Rare Disorders. (2023, November 20). https://rarediseases.org/rare-diseases/erythromelalgia/

Loureiro MP, Novais PM, Paulin JAN, de Almeida DB, de Lemos AN. Endoscopic lumbar sympathectomy as a treatment option for primary erythromelalgia – case report and review. J Vasc Bras. 2023 Mar 10;22:e20220095. doi: 10.1590/1677-5449.202200952. PMID: 36950140; PMCID: PMC10026352.

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

Skeik N, Rooke TW, Davis MD, Davis DM, Kalsi H, Kurth I, et al. Severe case and literature review of primary erythromelalgia: Novel SCN9A gene mutation. Vasc Med. 2011 Nov 9.

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