Erythromelalgia
Complex Regional Pain Syndrome
Information on erythromelalgia
Information about Spondylolisthesis
Information about Complex
Regional Page Syndrome
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for a detailed medical explanation of erythromelalgia.
My experience of EM and CRPS
What is Erythromelalgia (EM)?
Erythromelalgia is a rare condition that can cause redness and pain in all parts of the body, but most frequently the feet and hands (extremities).
It is characterized by intense, burning pain, severe redness (erythema), and increased skin temperature that may be episodic or almost continuous in nature. Although Erythromelalgia typically affects both sides of the body (bilateral), it may sometimes involve only one side (unilateral).
The disease course may be extremely variable from case to case. For example, in some individuals, symptom onset may be gradual, with the condition potentially remaining relatively mild for years. However, in others, it may have a sudden onset, possibly spreading and becoming severe over weeks.
The specific underlying cause of erythromelalgia remains unknown. However, the condition is thought to result from vasomotor abnormalities or dysfunction in the normal constriction and dilation of the diameter of certain blood vessels, leading to abnormalities of blood flow to the extremities. Erythromelalgia may be an isolated, primary condition or occur secondary to various underlying disorders.
The Erythromelalgia Association raises funds for research on EM. It offers education and support to all those affected by EM and strives to increase awareness about EM.
Erythromelalgia Symptoms
If you have been diagnosed with EM, symptoms may include hands, feet, face, ears, or other body parts that are very red to purple in color, may include oedema or swelling, may be extra warm or hot to the touch, and cause burning pain or deep aching. The intensity of the symptoms varies substantially from person to person.
Less frequently, EM symptoms may also appear in the face, ears, knees or other parts of the body. The intensity of the symptoms varies from patient to patient and often season to season. Some patients notice a continual burning pain while others are troubled with "flare-ups" or episodes of the condition lasting from minutes to days in length. It is very possible to have EM flaring on the face, the ears or nose, torso, or on other body parts in symmetrical or asymmetrical patterns. There have been rare reports of EM symptoms affecting internal organs as well.
As the etiology of Raynaud's Syndrome and Erythromelalgia seem to be closely related, some patients will have both. While it is not that uncommon for a patient to have both conditions, it is rare to see both conditions flare at the same time.
Environmental factors can make the symptoms of EM more severe, especially warm air temperatures. Heat intolerance and relief with cooling are hallmarks of EM. Additionally, some TEA members have found that foods, spices (monosodium glutamate), beverages (particularly alcohol) and some drugs can aggravate EM symptoms. Some patients experience EM "flaring" when the affected extremity is in a dependent position (hanging down) and relief may be instant when the patient can elevate the affected limb.
Facts about Erythromelalgia
EM is a rare disorder of unknown cause that can occur at any age, and affects both genders equally, and can present symptoms on the face, ears, torso, and other body parts symmetrically, or asymmetrically, although it is most commonly found in the extremities.
EM most frequently develops spontaneously and is therefore considered a primary disorder (meaning that there is no other underlying disease that is causing the EM.)
Sometimes EM develops secondarily to medical conditions such as neurologic, autoimmune or blood disorders (lupus, polycythemia vera, and multiple sclerosis are examples. Therefore it is very important to eliminate the possibility that you have any of these conditions at the time of diagnosis.)
People that first contract EM prior to age 25 or so, usually have primary EM and can trace its inheritance.
The causes of EM are not known, however, in recent years new discoveries from research centers in Europe and the United States have shed new light on its etiology.
Infrequently EM can develop from an injury, surgical procedure, or illness. Both the blood vessels and the nervous system are usually involved in EM.
Today, several treatments are available to control the pain, swelling, and redness of EM. Remissions are possible, but infrequent.
With the help of the members of The Erythromelalgia Association (TEA), their doctors, and future research, control of EM symptoms is entirely feasible.
Medical Explanation and effects on lifestyle
The following medical description may help you and your physician better understand this condition.
Jay Cohen, MD, Chairman of TEA’s Medical Advisory Committee, defines Erythromelalgia in the following way: “EM results from a maldistribution of blood flow with some areas not getting enough blood and calling for more. The extra blood gets through other open vessels, including arteriovenous anastomosis, that is, the connection between the arterial and venous systems. This vicious cycle continues to worsen until the overall appearance of the skin is of too much blood flow. Vasodilating drugs sometimes work to open the shut vessels. When that happens, the vessels stop calling for more blood and the overall system may normalize or at least improve its function.”
Dr. Cohen further says: “even mild erythromelalgia can greatly affect normal functioning and quality of life. Patients avoid warm weather and limit their activities to cool or air conditioned locations. Some move to cooler climates ... many patients cannot wear socks or closed shoes even in winter. ” When patients are severely affected, they can be house bound because of the continually painful symptoms. Elevation of the affected limbs becomes necessary and normal life is disrupted. Several cases of bed confinement due to unrelenting EM symptoms are known.
Treatments
Though there is no single treatment known for EM, there are several medications that are usually helpful in controlling the symptoms. Naturally, individual responses can vary substantially, and sometimes, maximum benefits are obtained by a combination of medications. Some people with EM are unusually sensitive to the effects of medications and should be started at very low doses.
In his article, “Erythromelalgia: New theories and therapies”, Dr. Cohen discusses a wide variety of possible approaches including the use of topical treatments, oral medications, drug infusions, surgical intervention, medical devices and non-medical therapies. In addition to medical treatments, there are things that an individual can do which will help maintain a more comfortable daily life. Elevation of the feet, to either above the heart, or level with the hips, each patient's needs may differ. Stay in a consistent and comfortable environment, air conditioning or at least a fan can help. Moderate exercise can be very important. Many people report that without frequent exercise, their EM symptoms are generally worse.
A strong word of caution about the use of cold water soaking and any drastic changes in air temperature: Though soaking your feet in cold water will make them feel better for a limited time, it can create many other serious problems. They include tissue breakdown of the skin, infections, reactive flaring and severe ulcers that can take many months to cure. Contrary to outdated medical information, this method of treatment is not advisable. Also note that even slight changes (5-10 degrees) in air temperature, hot or cold, may also create reactive EM flaring for some patients over the course of several hours.
Erythromelalgia Support
TEA has many members around the world, yet we stay in touch by several methods. We publish a newsletter filled with ideas and news from the general membership and Board of Directors on a quarterly basis. For those with Internet access, TEA also hosts this web site at www.erythromelalgia.org with new and informative articles about EM, back issues of the newsletter, and helpful links to learn even more about EM.
All of these resources and services have been very helpful to EM sufferers. In the years since TEA's formation, we have led the way in obtaining and organizing information about EM from around the world. Our members have participated in important medical surveys, and we have contributed to the publication of a major medical article that explains how to diagnose and treat Erythromelalgia (The Journal of the American Academy of Dermatology, November 2000), so that doctors and patients will know what to do about EM.
About The Erythromelalgia Association
Currently TEA has over 400 members of all ages, residing in many different countries. Originating from a handful of EM sufferers joining together to try and help others with this rare condition, TEA was created, and is still operated by volunteers, including our Officers and Board of Directors. TEA is advised and assisted by a Medical Advisory Committee made up of a number of doctors exceptionally experienced in EM research and working with EM patients.
T.E.A.'s Mission
TEA has been formed to accurately promote understanding of the symptoms and conditions of Erythromelalgia. We are committed through our experiences and research to maximize the effectiveness of health outcomes, including the quality of life, and to optimize the awareness of medical practitioners, patients and their families.
T.E.A.'s Main Objectives are...
To provide emotional support to those diagnosed with EM and their families.
To foster communication among members by sharing personal experiences relating
to EM.
To stress the importance of research into the causes, effects, diagnostic and
treatment options of EM within specific medical communities.
To cooperate and work closely with medical organizations currently involved
in finding new treatments for EM sufferers
To increase public awareness of EM and to stress the need for special accommodations
for those affected by the condition.
To create a global awareness where research information is readily available
to anyone involved in finding EM treatments and potential cures.
My experience and living with Erythromelalgia
My experience of EM can be summarised as follows:
1990: very severe attack of cellulitis, with hallucinations and blood poisoning, and breakdown and bleeding and seepage of 13-year old Achilles Tendon operation scars. Swelling became very intermittent but was not too bothersome for 4-5 years through my university studies.
1995: at a social event, summer heat was excessive, and gross swelling of my feet and ankles began. They showed poor recovery times, with skin breakdown (see below for full symptoms)
1999: tethered spinal cord at L5-S2 began deteriorating. EM worsened, but without knowing the reason for it, no treatment was sought, no cooling mechanisms used, and it seemed to be seasonal, confined to the winter (with central heating making it worse)
2000/01: situation continued about the same, with regular flare-ups, regardless of the seasons
July 2001: detethering of spinal cord. I made a good recovery considering the degree of tethering. I was back at full-time work after 14 weeks
October 2001: massive deterioration in EM status. From July 2001 to date, the emotional stress of coping with SBO/TCS and EM was overwhelming, debilitating, destructive and all-enveloping. To add the EM to the chronic conditions mix was particularly distressing and frightening.
June 2003: diagnosed myself with EM after a vascular surgeon dismissed me abruptly, walking out of his office without a word, and said there was no reason to call the gross swelling and burning pain "Erythromelalgia", and nothing could be done. I felt that no-one believed me and that the vascular surgeon felt I was imagining my symptoms.
This dismissal by the Vascular surgeon galvanised me into doing further detailed internet searching. After returning home from his clinic in floods of tears, distress and desperate for relief from the dreadful pain and burning feet from a flare-up of both EM and TC symptoms, I began my online searches into erythromelalgia.
In summary, EM is a hypersensitivity to hear and is sometimes called the opposite of Raynaud's Disease. My EM is caused by neurological damage to the autonomic nervous system, subsequent to the original spinal cord tethering. My symptoms affect my feet and ankles, which means any relief from the EM symptoms is hard to find, and the symptoms are hard to conceal from others. My symptoms include:
The cardinal diagnostic factors are that relief is obtained by cooling and elevation. I keep mine under some sort of control most of the year during the working day by having a fan in my office and sitting by it, with the jet of air focussed on my legs and feet, as long as I can. Going to the cinema, theatre, restaurants, and heated shops in winter are activites that are virtually impossible, unless I am prepared to cope with the consequences - gross swelling and pain. After a flare-up, which is very frequent in the winter months due to the central heating, to recover for the next day's work or activities, I usually lie on the floor in my cool house and bring the swelling down by using Aircast braces to disperse the oedema.
EM is a rare disease and not much is known about its treatment options, due to the varying factors and reasons for its occurrence. Vasodilators and vasoconstrictors are used widely, with varying degrees of success. In my case I found it hard, almost impossible, to use beta-blockers due to their side effects. I am very sceptical about any other medication to treat EM which is due to neurological damage, rather than metabolical or vascular.
However please see the Acupuncture
page to see how this may help in the alleviation of the worst signs and symptoms
of erythromelalgia and CRPS