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Frequently Asked Questions

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

What is erythromelalgia?

What parts of the body can be affected?

How can you differentiate erythromelalgia pain from other neurological pain?

How do I find out if I have Erythromelalgia?

What is the underlying cause of erythromelalgia?

What medical treatments are effective to reduce the symptoms of erythromelalgia?

What demographic and how many people have erythromelalgia?

How is EM diagnosed?

Are there other organizations that have information about this condition?

Do you have photos of what a flare looks like in actual people with erythromelalgia?

How can I meet other people who have the symptoms or have been diagnosed with erythromelalgia?

What is being done to find a cure?

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

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

Is Erythromelalgia a recognized disorder?

What is the origin of the term 'erythromelalgia'?

What disorders and diseases are similar to erythromelalgia and how can I find out about them?|

Can I get disability compensation for an erythromelalgia diagnosis?
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What is erythromelalgia?

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.

What parts of the body can be affected?

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.

How can you differentiate erythromelalgia pain from other neurological pain?

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 ice and water soaking is strongly discouraged as ice and water submersion can damage the skin.]  The characteristic of cooling bringing relief to EM sufferers pain is a hallmark trait of EM.

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.

Go to FAQ How is EM diagnosed? for further information

What is the underlying cause of erythromelalgia?

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.

What medical treatments are effective to reduce the symptoms of erythromelalgia?

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 Cymbalta help still others.  Combinations of drugs also have been reported effective. For instance, Lyrica and Cymbalta, at the lowest possible dosage, have been reported to be more effective than either drug by itself.

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.

What demographic and how many people have erythromelalgia?

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.

How is EM diagnosed?

Erythromelalgia can be diagnosed through observation and interview of the patient. A doctor who has knowledge of erythromelalgia (or the symptoms of autonomic nervous system dysfunction), can make a diagnosis of EM by asking pertinent questions of the patient and observing the affected body parts. The doctor will be looking at "tell-tale" signs of EM, such as, hot feet, burning pain and/or redness occurring in warm or increased temperatures, after exercise or extended period of time on feet and worsening in the evening/night time hours.

Another critical element of the diagnosis of EM is how the person can attain relief from the pain and discomfort: through elevation and cooling of the involved extremity (hands, feet, toes, etc.) The patient must tell the doctor about their method of attaining relief and any other affect the symptoms have on their lifestyle.

When a doctor does not have knowledge of EM . . .

Since EM is a rare disease, many, many doctors will have never seen the symptoms of EM in all the years they have been practicing and never read or heard about EM.

Therefore, the patient should be prepared for these circumstances by bringing a copy of:

Photos of actual flares:

    Before a flare                               Hands during a flare 
    During a flare
    Hands during a flare
    During a flare     Nose, cheeks, chin, 
ears flaring

 

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 

How can I meet other people who have the symptoms or have been diagnosed with erythromelalgia?

TEA hosts a ‘member directory’ on our website (under the Support tab) for members who would like to meet others with EM.  You can add your name to the directory when you register to be a member.
TEA also offers a Networking Program for those who are not online.  You can make a request at our Member Services phone messaging system or by emailing Member Services to find people in your area. 

There are at least five Facebook pages (not affiliated with TEA) that have both closed and open groups where users can exchange treatment, symptom and coping experiences.  Just go to the Search bar at the top of the Facebook page.

What is being done to find a cure?

There is currently no known cure for EM.  However, some patients have diligently pursued treatments that have made some improvement in their day-to-day lives by reducing symptoms to some degree.  On rare occasions, remission has occurred after use of a particular drug or treatment.  Such occurrences are described in articles found on this website under Articles.

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 difficult 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.

Is Erythromelalgia a recognized disorder?

While some doctors may not be very knowledgable about erythomelalgia, they all will be able to find it listed in the billing codes. Erythromelalgia and erythermalgia are listed under neurological disorders and is identified by the number 443.82 in the ICD-9 code list as of October 2005. Many searchable locations house articles and information on this disorder. Erythromelalgia is found in Medline, National Organization of Rare Disorders, and many more.

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)

What is the origin of the term 'erythromelalgia'?

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.

What disorders and diseases are similar to erythromelalgia and how can I find out about them?

Click for links to other similar or underlying disease informational websites

Can I get disability compensation?
An explanation of the U.S. disability benefit programs appeared in the winter 2014-2015 issue of FootSteps.
These are links to information about disability compensation programs in these countries.

 




 

 


CONDITIONS WITH SYMPTOMS SIMILAR OR OVERLAPPING WITH ERYTHROMELALGIA:

 

Complex Regional Pain Syndrome (CRPS) also known as Reflex Sympathetic Dystrophy (RSD)

http://www.rsds.org/index2.html

 

Chronic Inflammatory Polyneurpathy (CIDP)

http://www.gbs-cidp.org/

 

Dysautonomia also known as Autonomic Neuropathy

http://www.fightdysautonomia.org/Default.aspx?tabid=36

 

Fabry's Disease

http://www.fabry.org/fsig.nsf/Pages2/HomePage

 

Fibromyalgia

http://fibrohugs.org/

 

Marfan Syndrome

http://marfan.org/

 

Peripheral Neuropathy

https://www.foundationforpn.org/

 

Pheochromocytoma

http://www.pheochromocytoma.org/index.html

http://pheochromocytomasupportboard.yuku.com/

 

Raynaud's Disease or Phenomenon

http://www.raynauds.org/

 

Systemic Lupus

http://www.lifewithlupus.org/

 

Burning Mouth Syndrome
go4hope.org

 

Vasculitis

http://www.vasculitisfoundation.org/support

 

CHRONIC PAIN

http://www.theacpa.org/default.aspx

http://www.painconnection.org/support/index.html

 

FURTHER DESCRIPTIONS/EXPLANATIONS:

http://ghr.nlm.nih.gov/condition/erythromelalgia

ERYTHROMELALGIA SUPPORT GROUP:

 http://health.groups.yahoo.com/group/EM/

http://www.livingwitherythromelalgia.org/ 

GENERAL SUPPORT GROUP SITES:

www.puttingrarediseasespatientsfirst.org

www.experienceproject.com

www.dailystrength.org


DISABILITY COMPENSATION PROGRAM INFORMATION & LINKS
An explanation of the U.S. disability benefit programs appeared in the winter 2014-2015 issue of FootSteps. 
These are links to information about disability compensation programs in these countries.

 

CONDITIONS WITH SYMPTOMS SIMILAR OR OVERLAPPING WITH ERYTHROMELALGIA:

 

Complex Regional Pain Syndrome (CRPS) also known as Reflex Sympathetic Dystrophy (RSD)

http://www.rsds.org/index2.html

 

Chronic Inflammatory Polyneurpathy (CIDP)

http://www.gbs-cidp.org/

 

Dysautonomia also known as Autonomic Neuropathy

http://www.fightdysautonomia.org/Default.aspx?tabid=36

 

Fabry's Disease

http://www.fabry.org/fsig.nsf/Pages2/HomePage

 

Fibromyalgia

http://fibrohugs.org/

 

Marfan Syndrome

http://marfan.org/

 

Peripheral Neuropathy

https://www.foundationforpn.org/

 

Pheochromocytoma

http://www.pheochromocytoma.org/index.html

http://pheochromocytomasupportboard.yuku.com/

 

Raynaud's Disease or Phenomenon

http://www.raynauds.org/

 

Systemic Lupus

http://www.lifewithlupus.org/

 

Burning Mouth Syndrome
go4hope.org

 

Vasculitis

http://www.vasculitisfoundation.org/support

 

CHRONIC PAIN

http://www.theacpa.org/default.aspx

http://www.painconnection.org/support/index.html

 

FURTHER DESCRIPTIONS/EXPLANATIONS:

http://ghr.nlm.nih.gov/condition/erythromelalgia

ERYTHROMELALGIA SUPPORT GROUP:

 http://health.groups.yahoo.com/group/EM/

http://www.livingwitherythromelalgia.org/ 

GENERAL SUPPORT GROUP SITES:

www.puttingrarediseasespatientsfirst.org

www.experienceproject.com

www.dailystrength.org


DISABILITY COMPENSATION PROGRAM INFORMATION & LINKS
An explanation of the U.S. disability benefit programs appeared in the winter 2014-2015 issue of FootSteps. 
These are links to information about disability compensation programs in these countries.

 


CONDITIONS WITH SYMPTOMS SIMILAR OR OVERLAPPING WITH ERYTHROMELALGIA:

 

Complex Regional Pain Syndrome (CRPS) also known as Reflex Sympathetic Dystrophy (RSD)

http://www.rsds.org/index2.html

 

Chronic Inflammatory Polyneurpathy (CIDP)

http://www.gbs-cidp.org/

 

Dysautonomia also known as Autonomic Neuropathy

http://www.fightdysautonomia.org/Default.aspx?tabid=36

 

Fabry's Disease

http://www.fabry.org/fsig.nsf/Pages2/HomePage

 

Fibromyalgia

http://fibrohugs.org/

 

Marfan Syndrome

http://marfan.org/

 

Peripheral Neuropathy

https://www.foundationforpn.org/

 

Pheochromocytoma

http://www.pheochromocytoma.org/index.html

http://pheochromocytomasupportboard.yuku.com/

 

Raynaud's Disease or Phenomenon

http://www.raynauds.org/

 

Systemic Lupus

http://www.lifewithlupus.org/

 

Burning Mouth Syndrome
go4hope.org

 

Vasculitis

http://www.vasculitisfoundation.org/support

 

CHRONIC PAIN

http://www.theacpa.org/default.aspx

http://www.painconnection.org/support/index.html

 

FURTHER DESCRIPTIONS/EXPLANATIONS:

http://ghr.nlm.nih.gov/condition/erythromelalgia

ERYTHROMELALGIA SUPPORT GROUP:

 http://health.groups.yahoo.com/group/EM/

http://www.livingwitherythromelalgia.org/ 

GENERAL SUPPORT GROUP SITES:

www.puttingrarediseasespatientsfirst.org

www.experienceproject.com

www.dailystrength.org


DISABILITY COMPENSATION PROGRAM INFORMATION & LINKS
An explanation of the U.S. disability benefit programs appeared in the winter 2014-2015 issue of FootSteps. 
These are links to information about disability compensation programs in these countries.

 


Can I get disability compensation?
Can I get disability compensation?