Erythromelalgia

Erythromelalgia (pronounced Eh-ree-throw-mel-al-ghee-a) is a rare disorder that causes episodes of 'burning' pain. It is similar to Raynaud's in that it can affect the extremities but with Erythromelalgia, (EM) the sensation can also affect the arms, legs and face.

Symptoms of Erythomelalgia (EM)

Like Raynaud's, symptoms become apparent during an attack- or as it's known with Erythromelalgia- a 'flare-up':

  • Redness - caused by excessive blood flow to the affected area. The medical term for this is erythema.
  • Hot to the touch.
  • Pain - discomfort may range from mild tingling to severe burning. Others may feel sensations such as pins and needles or itching.

Other symptoms may include:

  • Swelling - the increased blood flow may cause a build-up of fluid in the affected body part. The medical term is edema
  • Change in perspiration - some people with erythromelalgia can sweat less (hidrosis) in the affected body part while others may sweat more (hyperhidrosis)
  • Purple discoloration and/or cold to the touch - some may experience cold and/or bluish skin when they are not experiencing a flare-up. This may be related to the narrowing of the blood vessels, known as vasoconstriction Some people may find that their symptoms appear on the face, ears, knees or other parts of the body.

What causes Erythromelalgia?

For most people with the condition, the cause is unknown but some underlying cause may be found such as a medical condition or faulty gene.

Underlying causes include:

  • Having abnormally high levels of blood cells
  • Either too many platelets in the blood or too many red blood cells
  • Damage to the peripheral nervous system
  • The network of nerves outside the brain and spinal cord
  • Multiple Sclerosis
  • A disease of the nerves in the brain and spinal cord
  • An autoimmune condition
  • Such as lupus, rheumatoid arthritis or scleroderma

What causes flare-ups?

Warm temperatures seem to be the most frequent trigger, something which is especially problematic for those with Raynaud's. Others have found that spices and alcohol can also make the symptoms worse. Flares may come and go or be continuous.

What are the different types of EM?

Primary - Primary EM is not caused by an underlying disease. It includes both inherited and idiopathic EM.

Secondary - Cases of EM that are caused by another disease or condition are called secondary EM. The most common causes of secondary EM are:

  • Essential thrombocythemia, a myeloproliferative blood disorder that causes increased production of platelets
  • Polycythemia vera, a myeloproliferative blood disorder that causes an increased production of blood cells, primarily red blood cells
  • Thrombocytopenia, a blood disorder that involves a low platelet count
  • Peripheral neuropathy, including diabetic neuropathy
  • Autoimmune diseases, such as lupus, vasculitis, rheumatoid arthritis, and scleroderma
  • Nerve damage due to injuries or other conditions, such as carpal tunnel syndrome, sciatica, and frostbite

In some cases, when the underlying condition is treated, EM symptoms will improve or resolve completely.

If you experience any of the above symptoms then please speak with your GP or consultant regarding the best way to manage your symptoms.

This information has been produced in partnership with NHS Choices and The Erythromelalgia Association (TEA)

Can it be treated?

Intravenous infusion In some cases, when pain has not been controlled by medication taken by mouth (orally), an intravenous infusion (when medicine is given directly into your bloodstream via a drip) may be used. Lidocaine – a local anaesthetic that blocks sodium channels and can help nerve-related pain – may be given this way, but how long it works for varies. Your doctor will explain the procedure to you and how you should prepare for it.

Medication taken by mouth

A number of different medications have shown potential in relieving symptoms, although no single drug helps everyone. Different treatments often need to be tried under the supervision

of experienced clinicians, and combinations of different medicines are sometimes needed. Your doctor will discuss treatment options with you, as this will also depend on the type of erythromelalgia you have.

Drugs used for other types of nerve pain may reduce symptoms. This includes anti-epilepsy drugs such as gabapentin, or low doses of tricyclic antidepressants such as amitriptyline, which can be effective in treating pain caused by increased sensitivity or damage to the nervous system.

Drugs 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).

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