The Erythromelalgia Association

Photos of EM Sufferers

Click images to enlarge

Feet flaring

Before a flare

During EM flare

Hands flaring

Flaring can affect

the nose, chin,

ear(s), and cheeks

Simultaneous

Raynauds/EM

What is EM?

Erythromelalgia is a rare and frequently devastating disorder that typically affects the skin of the feet or hands, or both, and causes visible redness, intense heat and burning pain.

The term erythromelalgia describes the syndrome: erythros (redness), melos (extremity) and algia (pain). An alternate name is “erythermalgia” that emphasizes the thermos (heat) – an essential part of the syndrome.

While usually affecting the lower extremities (legs and feet) and upper extremities (arms and hands) other body parts like faces or just ears or the nose may be involved. It usually affects both sides of the body, but can affect just one.

The associated pain and burning sensations can be extremely severe. People with EM often make major adjustments to their lifestyles to avoid flare-ups. Even in mild-to-moderate cases, normal functioning such as walking, standing, working, socializing, exercising, and sleeping may be impaired.

Precipitating factors include warm temperatures and even mild exercise. Cooling the hot body parts relieves the pain, as does elevating the affected areas. [The use of icy water is strongly discouraged as ice can damage the skin. Using a fan or cool water is suggested instead.]

Approximately five percent of those with EM have an inherited condition that research has shown to be caused by genetic mutations. These people usually have other members of their families with the syndrome.

The cause of EM is unknown in the vast majority of cases. Peripheral neuropathies underlie many, and in some cases EM may be secondary to other disorders like the blood disease polycythemia.

A number of different medications have been shown effective in relieving symptoms of EM. However, no one drug helps everyone. Traditional over-the-counter pain medications or stronger prescription drugs help some. Anticonvulsant drugs like Neurontin and Lyrica help others. Certain antidepressants like Effexor help still others.

It is recommended that people with EM find a doctor willing to help them pursue a trial-and-error course of treatment. Since some people may be highly sensitive to drugs, initial doses of new medications should be very low.

Recent research in the U.S. found the incidence of EM (the number of people a year diagnosed with EM) to be 1.3 per 100,000. The rate for women was higher – 2.0 per 100,000 per year – than men, which was just 0.6. The median age at diagnosis was 61.

These rates were five times higher than those estimated by an earlier Norwegian study – the only other known research describing EM incidence.

FAQ

Frequently Asked Questions

The information contained below is intended for generalized and educational information only. Please seek further information or diagnostics from your doctor.

Are there other organizations that have information about this condition?

Yes, there are several that may offer additional information. One valuable resource is the National Organization of Rare Disorders (NORD). They offer a report about the basics of erythromelalgia and can be reached at: www.rarediseases.org

TEA offers a Networking Program to help put people in touch with others who have EM. To join this program you will need to be a member of TEA. Go to Become a Member on the Navigation bar on the home page to join TEA now.

What is being done to find a cure?

There is no simple cure for EM that we know of. However, many patient have diligently pursued treatments that have significantly reduced or eliminated any discomfort or symptoms. The cause of EM is not proven as yet, therefore, a cure has not been identified. Remissions have occurred after several different courses of treatments and articles describing these are available from TEA for TEA members only.

Is there a cure for Erythromelalgia?

There is no simple cure for EM that we know of. However, many patients have diligently pursued treatments that have significantly reduced or eliminated any discomfort or symptoms. The cause of EM is not proven as yet, therefore, a cure has not been identified. Remissions have occurred after several different courses of treatments and articles describing these are available from TEA for TEA members only.

What can I do about my symptoms... today?

Elevation of the affected part usually works well, also cooling of the area is generally effective temporarily. However, be aware that icing and soaking in cold water can cause permanent tissue damage when used for long and consistent periods of time. Even temporary extreme changes in temperature can cause a cycle of flaring that is difficult to break. Please consult your doctor prior to undertaking ANY treatment or therapy.

I've been to my doctor and no diagnosis was made, now what do I do?

Don't give up. The diagnosis of any neuropathy or syndrome that is considered a rare disorder is very di icult to make. EM has no test or conclusive evidence that facilitates a diagnosis, and many doctors may never have heard of EM or seen a case to compare yours with. Try visiting with a specialist in neuropathies, pain disorders, dermatology, etc. Most major medical centers should be able to help you locate a doctor with experience in diagnosing and treating EM. More dead ends? Contact us, we may be able to help you connect with one. We also can suggest reference materials for your doctor to use in helping treat your condition.

How do I find out if I have Erythromelalgia?

Erythromelalgia generally affects the feet and hands (although it can affect the face and ears as well as other parts of the body). There is usually a warmth and discomfort that is often characterized as a burning sensation. This pain is almost always accompanied by redness and/or swelling. The symptoms are generally made worse by putting the extremity below the level of the heart and by external warmth. The pain is relieved by elevation. The cardinal feature of the disorder is that the symptoms may temporarily be relieved by cooling of the affected area. A medical doctor may be able to give you an official diagnosis.

Is Erythromelalgia a recognized disorder?

ICD-10 I73.8 Other Specified Vascular diseases

ICD-9 443.82 Erythromelalgia

OMIM 133020 (NCBI - Online Mendelian Inheritance in Man)

MeSH D004916 (National Library of Medicine - Medical Subject Headings)

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