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Erythromelalgia is a rare disease that is often overlooked. There are too few affected patients. Writings on the subject are consequently rare. It is therefore important to convey information to affected patients, their families and to all others who would be interested in learning more on the subject.

What is erythromelalgia?

Let us first dissect the word erythromelalgia. The first part "erythro" indicates redness, "mel" means limb (s) and "algia" stands for pain.

Erythromelalgia, also called Mitchell's disease in honor of the doctor who was the first to mention the presence of this disease in 1878, generally affects the extremities of the body such as feet, heels, toes, knees, legs but also more rarely the hands, elbows, face, nose and ears.

Flares can range from a few minutes to several hours. They are characterized by a feeling of warmth, redness of the skin (like a sunburn) and swelling of the affected limbs. Depending on the level of the attack, pain may be mild and consist of tingling. However, when the attack is severe, pain is stronger and resembles an intense burn.

Here are some factors that can trigger flares:
• ambient temperature and/or direct exposure to sunlight;
• prolonged standing positions;
• physical efforts such as walking or running;
• hot water flowing over the skin, for example by washing hands or taking a shower.

Often the erythromelalgia sufferer will try to relieve its pain with compresses of cold water or ice. This tactic seems to work at first, but over time, it worsens the symptoms. Raising affected limbs and resting them is often more effective.

Cause

There are two types of erythromelalgia: primary and secondary.

Primary erythromelalgia

When the cause of the disease is unknown, it is assigned to the primary category. There is also an inherited form of the disease, which usually begins at birth or during childhood.

Secondary erythromelalgia

In the case of secondary erythromelalgia, the onset of the disease can be linked here to myeloproliferative disorders or to the taking of certain drugs such as nifedipine or bromocriptine.

Diagnostic

There is no known test to diagnose erythromelalgia, no blood test. It is therefore necessary to proceed by elimination because there are other diseases which have similar symptoms such as complex regional pain syndrome, rheumatoid arthritis, Fabry disease, hyperperfusion in Raynaud's syndrome, mercury intoxication, peripheral neuritis, bacterial cellulitis, mushroom poisoning, obliterating thromboangiitis or obliterating arteriosclerosis.

Doctors also recommend taking notes and pictures during flares to help them observe the evolution of symptoms.

Treatment

Some forms of erythromelalgia can be easily treated with lower dose of aspirin. But when this means fails, there are no other alternatives at the moment. Doctors then try to prescribe medication to lessen the pain.

The information contained on the erythro.ca website is for educational purposes only and is not intended to replace the advice of a physician.

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