TEA has compiled a list of answers to frequently asked questions regarding EM. The information contained within the FAQs is intended for generalized and educational information only.
What is erythromelalgia?
Erythromelalgia (EM) is a rare neurovascular condition that most commonly affects the feet, but may also occur in the hands, face, or other parts of the body. The term “neurovascular” indicates that both nerves and blood vessels are involved. The three hallmark symptoms of EM are redness, warm to hot skin, and burning pain. Additional symptoms may include swelling or changes in perspiration, and the skin may be cold and/or bluish purple when not actively flaring. EM flares may be intermittent or continuous, mild or severe, and are most commonly triggered by warmth, physical activity, or stress.
The term “erythromelalgia” derives from the Greek words erythros (redness), melos (limb or extremity), and algia (pain). An alternate name is “erythermalgia,” which incorporates the Greek word thermos (heat).
How common is EM?
According to a 2008 study conducted by Mayo Clinic, EM affects an estimated 1.3 people per 100,000 each year in the United States. A 2013 study from New Zealand suggests that the incidence of EM may be as high as 15 per 100,000. EM is considered a rare disease and is listed in the database of the National Organization for Rare Disorders (NORD).
What causes EM?
There are several types of EM. When EM is not caused by an underlying disease, it is called “Primary EM.” This includes both Inherited EM, which is hereditary and caused by mutations of the SCN9A gene, and Idiopathic EM, which has no identifiable cause. Idiopathic EM is the most common form.
Cases of EM that are caused by another disease are referred to as “secondary EM.” Common causes include blood disorders, peripheral neuropathy, autoimmune diseases, and nerve damage. In addition, some medications have been linked to the onset of EM. In some cases, when the underlying cause is addressed, EM symptoms will improve or resolve completely.
How is EM diagnosed?
The first step is seeing your primary care doctor, who may then refer you to a specialist. Specialists that diagnose and treat EM include neurologists, dermatologists, rheumatologists, and vascular physicians or surgeons. TEA maintains a list of doctors who have been recommended by members for treating EM.
There is no specific test for EM. Doctors diagnose EM by observing symptoms and by ruling out any other possible cause of your symptoms. They may also run tests to identify or rule out any possible underlying disease that could be causing your EM symptoms.
What is my prognosis?
In a Mayo Clinic study of patients with EM symptoms, approximately 30% got better, 30% got worse, and 30% stayed the same. In about 10% of those cases, the EM resolved completely.
How is EM treated?
There is no single “cure” for EM. While it is true that treating an underlying disease may sometimes bring complete relief, most cases of EM cannot be fully treated. In such cases, treatment must focus on relieving symptoms with medications and other techniques. It often takes a period of trial and error to find a treatment plan that works.
Treatment options include topical medications, oral medications, supplements, and surgical procedures, as well as alternative and mind/body therapies. For a detailed list of potential treatments, please see TEA’s Patient’s Guide.
Is there anything else I can do to relieve my symptoms?
Cooling the affected area typically provides some relief for an EM flare, and you may be tempted to soak your feet in cold water. This is a dangerous practice that can seriously damage the skin, as well as lead to nerve damage and rebound flaring. Ice packs should also be avoided.
There are a number of safe methods for cooling a flaring body part, such as directing a fan on the affected area, resting your bare feet on a cool tile or concrete floor, applying cool (not cold) gel packs to the affected body part, and elevating flaring feet. When utilizing a fan or cool gel packs, moderation is key. If used for more than a short period of time, these methods can potentially damage skin and make your symptoms worse.
In addition to relieving painful symptoms, coping with EM also involves taking measures to prevent flares from occurring. To do this, you must first identify what triggers an EM flare for you, and then limit or modify those activities or situations. Four of the most common EM triggers are exercise, bathing, eating certain foods, and sleep. For some helpful lifestyle modification tips, please see TEA’s Patient’s Guide.
Why are my feet/hands sometimes burning hot and other times painfully cold?
It’s possible that you may also have Raynaud’s phenomenon, a condition that sometimes coexists with EM. With this disease, fingers or toes turn white or blue in response to cold temperatures or emotional stress. Some people describe Raynaud’s as the “opposite” of EM. If you are having these symptoms, talk to your doctor about possible treatment options.
How can I connect with other people who also have EM?
In coping with your EM, you may be faced with feelings of isolation, particularly if you are housebound. It can be very helpful to connect with people experiencing the same symptoms as you.
TEA provides a Member Directory 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. (For those who are not online, TEA offers a Networking Program to help find others with EM in their area. You can make a request by calling Member Services at 610-566-0797.)
Online groups are a great way to interact with others who can relate to your experiences with EM. Members may share treatments that have worked for them, offer tips on how to deal with EM-related challenges, and generally inspire each other to keep going in the face of a chronic, painful condition.
TEA hosts a Facebook page, which provides the latest EM news as well as an opportunity to connect with others through social media. In addition, there are several other Facebook pages and online support groups that are not affiliated with TEA (see Helpful Links). You can also find active groups by searching online.
Is EM a recognized disorder?
While some doctors may not be very knowledgeable about EM, they will be able to find it listed in the billing codes. Erythromelalgia and erythermalgia are listed under neurological disorders, and the condition 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/PubMed, National Organization for 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 disorders and diseases are similar to EM, and where can I find information about them?
Click here for a list of helpful links, including websites with information on similar or underlying diseases.
Am I eligible for disability compensation?
If the onset of EM has left you unable to work, you may be eligible to receive government benefits to help compensate for your loss of income. An explanation of the U.S. disability benefit programs appeared in the Winter 2015 issue of FootSteps.
What is being done to find a cure?
TEA encourages and funds research into effective treatments for all types of EM. Though EM is rare and has not been extensively studied, research into its causes, mechanisms, and treatments continues in both academic and commercial settings. At present, there is a growing interest in developing EM treatments, due in part to the belief that a drug designed to treat EM pain could work for many other kinds of pain as well. For the most up-to-date information on advances in EM research and treatments, see TEA’s In the News, Medical Articles, Research Timeline, and FootSteps Newsletters.