What is erythromelalgia?

Erythromelalgia is a rare distressing condition that is difficult to treat and primarily affects the feet and hands. It is characterized by the triad of redness, warmth and burning pain. The attacks of this conditions are periodic and last anywhere from minutes to days. Attacks will normally start with a simple itching, but then progress to intense pain and a burning pain. The foot or hand will become warmer, tender and swollen as well as take on a reddish colour. The attacks are very disabling and will have a significant impact on the person with an affect on the quality of life and the ability to carry out some of the normal activities of daily life.

There are two types of erythromelalgia. There is the primary type that tends to affect younger people and is most likely due to a hereditary genetic mutation that make nerves more excitable. The secondary type tends to affect older people and is due to an underlying medical condition such as diabetes, gout, some blood disorders or as a side affect to certain drugs. There are a number of conditions that are similar and doctors will need to differentiate it from them as part of the investigation. These include conditions like chronic pain problems, chilblains and burning foot syndrome.

The treatment of erythromelalgia is challenging as there is no one treatment that seems to help everyone and the response to treatment is quite variable between individuals. The first approach is to avoid situations that trigger an attack such as a warm bath, environmental triggers or certain foods. Most people with it do find relief from cooling with the use of air conditioning and cooling gels. Some do resort to immersion of the limb in cold water but this is not recommended as this can lead to maceration of the skin and perhaps chilblains and they may develop a secondary infection.

For the secondary type of erythromelalgia, management is usually directed at management of the underlying medical condition, and this can often be very helpful. For primary type of erythromelalgia the management of the pain is often much more challenging with the response to different medications being different between people. Often anti-inflammatory drugs are tried, but are often unhelpful. Drugs that block the channels in the nerves that make the nerves overactive are often tried and they do tend to help some. The anticonvulsant drugs such as gabapentin may help some. There are some topical drugs that some find helpful such as capsaicin. In the most severe cases sympathetic block or a sympathectomy which does involve surgery on the back may be undertaken. However, the results of these are mixed. As the pain is intense and the condition is difficult to manage and has such an affect on the daily life of those who have erythromelalgia, it is strongly recommended that all those with it have a consultation with a pain management specialist and clinical psychologists to help them cope with the symptoms and learn the strategies that are helpful for coping.