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Erythromelalgia

ERYTHROMELALGIA

What are the aims of this leaflet?

This leaflet has been written to help you understand more about erythromelalgia. It tells you what this condition is, what it is caused by, what can be done about it, and where you can find out more about it.

What is erythromelalgia?

Erythromelalgia (or erythermalgia) is a rare condition. It causes intermittent, painful swelling and redness of the feet or hands. Sometimes the legs and arms can be affected, or, less often, other areas such as the ears or nose. It usually affects both sides of the body. In ancient Greek, ‘Eythros’ means ‘red’ and ‘melalgia’ means pain in a limb.  Early-onset erythromelalgia starts before the age of 25, and late-onset develops between the ages of 40 to 60.

What causes erythromelalgia?

Erythromelalgia can be either the more common ‘primary’ form of unknown cause, or a rarer ‘secondary’ form, when it is linked to another illness. In the secondary form there may be too many platelets in the blood (e.g. in the blood disorders polycythaemia vera or essential thrombocytosis). Blood tests are usually requested at diagnosis and may be need to be repeated from time to time.

The secondary form can also be associated with other diseases including diabetes mellitus, rheumatoid arthritis, gout, human immunodeficiency virus and systemic lupus erythematosus.

Erythromelalgia may be due to problems with nerves running from the brain to blood vessels in the affected area, altering the circulation in the affected area and causing pain.

Is erythromelalgia hereditary?

Primary erythromelalgia can run in families and may be traced to a particular gene. However there is usually no family history of erythromelalgia.

What are the symptoms of erythromelalgia?

The symptoms of erythromelalgia are redness, increased skin temperature and a burning sensation at the affected site. Episodes may be intermittent or occasionally continuous and may last for a few minutes to several days. The onset of symptoms can be gradual or sudden. An itching sensation may precede the burning pain which can range from mild to severe and disabling. In some people ulcers can develop.

A flare up of erythromelalgia can be triggered by exposure to warmth, for example coming into a warm room, or by tight fitting clothing, or by exercise.

What does erythromelalgia look like?

During a flare, the affected area looks red or blue, mottled and swollen, and feels warm to the touch. In between episodes the skin can look normal.

How is erythromelalgia diagnosed?

Erythromelalgia may be diagnosed without tests by its signs and symptoms. Blood tests may be requested to look for an underlying medical condition.

Can erythromelalgia be cured?

There is no cure for erythromelalgia. Primary erythromelalgia can sometimes settle on its own.

How can Erythromelalgia be treated?

Unfortunately, responses to treatment vary. If an underlying medical condition has been found, treatment of this may improve the erythromelalgia.

Loose clothing, cooling measures and elevating the affected body part may relieve the burning pain. Prolonged submersion in water is not recommended.

Treatments that your clinician may use to help the pain of erythromelalgia include:

  • Aspirin.

  • Capsaicin cream to the affected area.

  • Anti-convulsant medications: e.g. Gabapentin, Pre-gabalin, carbamazepine.

  • Newer antidepressants:  e.g. venlafaxine, sertraline, fluoxetine, paroxetine.

  • Tricyclic antidepressants:e.g. amitriptyline.

  • Calcium channel blockers (e.g. nifedipine, diltiazem).

  • Intravenous infusions (through a drip) of nitroprusside, prostaglandin (iloprost) or lignocaine (lidocaine). Mexilitine tables work similarly to lignocaine, but can be difficult to obtain. A lidocaine patch applied to the skin may also help.

  • Nerve blocks or surgical sympathectomy (a procedure in which sympathetic nerve fibres are cut) have helped some, but were not beneficial in others. Sympathectomy can cause permanent severe side effects in some people.

Where can I get more information about erythromelalgia?

Web links to detailed leaflets:

http://dermnetnz.org/vascular/erythromelalgia.html

Support group for patients with erythromelalgia:

http://www.livingwitherythromelalgia.org/

For details of source materials used please contact the Clinical Standards Unit (clinicalstandards@bad.org.uk).

This leaflet aims to provide accurate information about the subject and is a consensus of the views held by representatives of the British Association of Dermatologists: its contents, however, may occasionally differ from the advice given to you by your doctor.

This leaflet has been assessed for readability by the British Association of Dermatologists’ Patient Information Lay Review Panel

BRITISH ASSOCIATION OF DERMATOLOGISTS

PATIENT INFORMATION LEAFLET

PRODUCED JULY 2014

REVIEW DATE JULY 2017

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