Erythromelalgia

Posted on A to Z of Medical Conditions

Erythromelalgia – so what is Erythromelalgia?

Erythromelalgia is a rare condition that causes episodes of burning pain and redness in the feet, and sometimes the hands, arms, legs, ears and face.Symptoms of erythromelalgia can begin at any age. Some people may have had it from early childhood, while some are only affected as adults.

Symptoms of erythromelalgia

The three main symptoms of erythromelalgia are heat, pain and redness in the skin.

The feet are most commonly affected, but the hands, arms, legs, ears and face can be too.

Picture of erythromelalgia in the hands

The pain can range from mild, with only a minor tingling feeling like pins and needles, up to a severe burning pain, which can be bad enough to make walking, standing, socialising, exercising and sleeping difficult. It can have a significant impact on work or school life.

People with erythromelalgia typically suffer episodes or “flare-ups” of pain lasting from a few minutes to days. The flare-ups usually start as an itching sensation, which worsens to pain, and tender, mottled red skin that feels warm or hot to the touch.

Other symptoms may include:

  • swelling of the affected body part
  • sweating in the affected area more or less than you usually would
  • purple discolouration when there is no flare-up

Triggers for erythromelalgia

Symptoms are usually triggered by an increase in body temperature.

This can happen:

  • after exercising
  • when wearing warm socks, gloves or tight shoes
  • after entering a warm room
  • when feeling stressed
  • when drinking alcohol or eating spicy food
  • when you’re dehydrated

Treatments for erythromelalgia

Elevation and cooling the skin

Cooling or elevating the affected part of the body may help relieve symptoms.

The skin can be cooled using a fan, cool water, a cool surface or cool gel packs.

However, avoid using ice or anything that’s too cold, and don’t soak hands or feet for a long time in cold water. This can lead to hypothermia or skin damage. And there’s also a risk the change in temperature may trigger a flare-up when the affected area warms up again.

Medications for the skin

Some medications that are applied directly to the skin (topical medication) have been found to help relieve the symptoms of erythromelalgia.

These may be in the form of creams, gels, sprays or patches. You may be prescribed a capsaicin cream or patch to make the heat receptors in your skin less sensitive. Lidocaine (a local anaesthetic) may also be prescribed in the form of a cream, gel or spray.

Your doctor will be able to give you more information about these medications and if they’re right for you.

You can also speak to your pharmacist about lidocaine creams.

Medication taken by mouth

A number of different medications taken by mouth (orally) may help to relieve the symptoms of erythromelalgia.

You may need to try several different medications, under the supervision of your doctor, before you find the one or the combination that works best for you.

Your treatment options will also depend on the type of erythromelalgia you have. Many treatments require referral to a specialist centre so benefits and potential side effects can be closely monitored.

The types of medication your doctor may prescribe include:

  • dietary supplements – such as magnesium, which can help open up your blood vessels
  • aspirin – only used for adults, not for children
  • anti-epilepsy drugs – such as gabapentin or carbamazepine
  • blood pressure drugs – medication to either open up your blood vessels and increase blood flow, or beta-blockers to help reduce blood flow, depending on the cause of your erythromelalgia
  • low doses of antidepressants – such as duloxetine, venlafaxine, amitriptyline or nortriptyline
  • prescription-only painkillers

Medications given via a drip

In some cases, when oral medication has not managed to control the symptoms, medicine may be given directly into the bloodstream via a drip (intravenous infusion). Lidocaine, a local anaesthetic that can help nerve-related pain, can be given this way. But how long it works for varies between people.

Your doctor will explain this procedure to you and how you should prepare for it.

Causes of erythromelalgia

In most cases of erythromelalgia, the cause is unknown.

However, it’s sometimes due to another underlying medical condition or a faulty gene inherited from a parent.

Other medical conditions

Erythromelalgia sometimes results from an underlying condition, such as:

It may also be caused by certain medications. Your doctor will be able to give you more information on this.

Genetic cause

In some people with erythromelalgia, the disease is caused by a faulty gene. Erythromelalgia can run in families when the faulty gene is passed down from a parent to their child (inherited).

The faulty gene causes changes in the way pain signals are delivered to the brain, increasing or strengthening them.

More information and support

Specialist centres in the UK

For children: Great Ormond Street Hospital for Children Pain Control Service

For adults: National Hospital for Neurology and Neurosurgery Pain Management Centre

Organisations that provide support

Information about you

If you or your child has erythromelalgia, your clinical team will pass the information on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

Find out more about the register.

Page last reviewed: 26/09/2017
Next review due: 26/09/2020

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Erythromelalgia – do you know the signs?

Erythromelalgia: This 77-year-old woman with longstanding polycythemia vera had a six-month history of increasingly prolonged bouts of redness, swelling, and burning pain in her extremities. The severity and sites of involvement varied with each episode. At presentation, she was unable to ambulate without assistance. by Herbert L. Fred, MD and Hendrik A. van Dijk - http://cnx.org/content/m14932/latest/

Erythromelalgia: This 77-year-old woman with longstanding polycythemia vera had a six-month history of increasingly prolonged bouts of redness, swelling, and burning pain in her extremities. The severity and sites of involvement varied with each episode. At presentation, she was unable to ambulate without assistance.
by Herbert L. Fred, MD and Hendrik A. van Dijk – http://cnx.org/content/m14932/latest/

Erythromelalgia is a rare disorder that causes episodes of burning pain and redness in the hands and feet, and sometimes the arms, legs, ears and face.  

Cooling the skin or raising the affected hands or feet may reduce the pain.

Symptoms can begin at any age. Some people with erythromelalgia may have had it from early childhood, while some are just affected in adult life.

How severe can it be?

People with erythromelalgia typically suffer episodes or flare-ups of severe pain lasting from a few minutes to days.

Flare-ups usually start as an itching sensation, which worsens to a severe burning pain and tender, mottled red skin.

The hands and feet are most commonly affected, usually on both sides of the body, but erythromelalgia can also affect the arms, legs, ears and face.

The pain can be severe enough to make walking, standing, socialising, exercising and sleeping difficult. It can have a significant impact on work or school life.

What are the triggers?

Symptoms are usually triggered by an increase in body temperature.

They can start after exercising, wearing warm socks, gloves or tight shoes, entering a warm room, being dehydrated, or even consuming alcohol or spicy food.

What is the cause?

For most people with erythromelalgia, the cause is unknown.

However, in some cases, an underlying cause may be found – usually a medical condition or a faulty gene (see below).

Underlying disease

Erythromelalgia sometimes results from an underlying disease, such as:

having abnormally high levels of blood cells – either having too many platelets in the blood (thrombocythemia) or too many red blood cells (polycythaemia)

damage to the peripheral nervous system – the network of nerves outside the brain and spinal cord (this damage is known as peripheral neuropathy)

multiple sclerosis (MS) – a disease of the nerves in the brain and spinal cord

an autoimmune problem – such as lupus or rheumatoid arthritis, where the immune system attacks the body’s own tissues

It may also result from certain medications, such as verapamil or nifedipine.

Genetic cause

In some people with erythromelalgia, the disease is caused by a change (mutation) in a gene. This may have occurred spontaneously or been inherited from a parent, meaning that erythromelalgia can run in the family.

Genes contain the genetic information, or the “instructions”, to make a particular protein in the body. Genetic mutations occur when DNA changes, altering the genetic instructions.

Erythromelalgia can be caused by changes in the SCN9A gene that provides the instructions for a sodium channel in pain nerves.

Changes in sodium channels can lead to them opening more easily and staying open longer than usual. This increases or amplifies pain signals.

It is not currently understood why the hands and feet are affected the most.

Can it be treated?

Cooling the skin and elevation

Cooling the skin with a fan or cold water, or raising the affected hands or feet may relieve the pain.

However, using ice or soaking the hands or feet for long amounts of time isn’t recommended, as this can lead to hypothermia or damage to the skin, causing ulcers.

There’s also a risk that the change in temperature will trigger a reactive flare-up when the hands or feet are removed.

Even though a cool environment is helpful in reducing pain during attacks, the use of cold baths or showers is generally discouraged because of potential skin damage.

Your doctor will discuss treatment options with you, as this will also depend on the type or erythromelalgia you have. Many treatments require referral to a specialist centre so that benefits and potential side effects can be closely monitored.

Products used for other types of nerve pain may reduce symptoms.

Products used for high blood pressure or Raynaud’s disease can encourage the blood vessels to widen, and may be beneficial in some types of erythromelalgia.

In adults, aspirin may relieve symptoms if the cause is an abnormally high number of blood cells (aspirin is not recommended for children).