Erythromelalgia: burning pain, redness and heat in the feet and hands

The name comes from Greek words meaning red and limb pain. BAD explains that ‘erythros’ means red and ‘melalgia’ means pain in a limb. It describes episodes of intense burning pain, painful swelling and redness of the feet or hands, sometimes the legs and arms, and less often other areas such as the ears or face. It usually affects both sides of the body, females are more commonly affected than males, and it can occur in all ethnic groups.

BAD says erythromelalgia is thought to be due to problems with the nerves that control the body’s blood vessels. As a result, blood flow to the affected area increases, which leads to pain, swelling and redness or skin darkening. That is why the affected skin feels hot and why warmth makes things worse.

The NHS lists the main symptoms as burning pain, which often starts as itching, redness (which can look different on brown or black skin) and warm or hot skin, with swelling and sweating more than normal as other symptoms. The pain can be constant or come and go, and a flare-up can arrive gradually or suddenly and last from a few minutes to a few days. The NHS adds that skin may feel cold after a flare-up and look blue or grey.

BAD describes the skin during a flare-up as red, blue, darker or mottled and swollen, and warm to the touch, with the skin often looking normal, mildly cool or occasionally paler between episodes. In some people ulcers can develop at the affected sites, and some people notice symptoms worsening at night because of the warmth of bedding.

The pain and swelling can cause significant psychological distress, according to BAD. The NHS likewise says that living with pain can be difficult and that cognitive behavioural therapy and counselling can help if symptoms are affecting mental health.

The NHS says it is often not clear what causes erythromelalgia. Sometimes it is caused by another condition or by a faulty gene inherited from a parent.

BAD sets out the two forms. Primary erythromelalgia is caused by a change in the pain nerves due to a change in one of your genes; it can occur at a younger age and in some cases symptoms start at puberty. It can run in families, with a one in two chance of an affected person passing it on to each child, although sometimes no family history is found. The rarer secondary form comes on later in life, usually between the ages of 40 and 60, and is usually linked to another illness. BAD says tests might be done to rule out causes such as medications, diabetes, lupus, HIV, bone marrow disorders and pregnancy.

Identifying personal triggers is a large part of day-to-day management. The NHS lists the following as things that raise body temperature and may set off a flare-up.

• Exercise.

• Warm or tight clothes and shoes.

• Warm temperatures, such as in hot weather.

• Stress.

• Drinking alcohol or eating spicy food.

• Not drinking enough fluids (dehydration).

The NHS says that if a GP thinks you may have erythromelalgia, they will refer you to a specialist, who will examine you, ask about your symptoms and may do tests including blood tests and genetic and genomic testing.

BAD adds that erythromelalgia may be diagnosed from the signs and symptoms without tests. Genetic testing may be considered if primary erythromelalgia is suspected, looking for changes in the sodium channel gene SCN9A, and blood tests may be requested if secondary erythromelalgia is suspected, to look for other diseases that could be causing it.

The NHS says treatment depends on the cause. If another condition is responsible, treating it can help. Most treatment aims to prevent flare-ups and can include medicines, creams, gels, sprays, tablets and medicines given into a vein, and rarely surgery. It also says that erythromelalgia can be difficult to treat and that you may need to try different combinations before finding what works for you.

BAD gives more detail on what specialists may use, and stresses that people sometimes need to try several medicines or combinations.

The NHS advice is to avoid triggers, wear loose clothing and shoes, drink plenty of fluids, try to manage stress, and cool the hands or feet with a fan or a cool, not cold, gel pack for a short period. Raising the hands or feet on a chair or pillow during a flare-up can also help. It says not to cool with cold water or ice, which can damage the skin, and not to drink alcohol or eat spicy food.

The NHS points to The Erythromelalgia Association as a source of further information and support.

The NHS says a GP who suspects erythromelalgia will refer you to a specialist, so the usual route into care starts with an appointment at your GP surgery. Because the condition is rare and can be difficult to treat, the specialist involved may be a dermatologist, a pain specialist or another doctor, depending on the likely cause.

People can also choose to see a specialist privately, usually after a GP or consultant referral letter. Whichever route you take, it helps to keep a short diary of when flare-ups happen, what you were doing and how warm it was, because triggers are a large part of the NHS advice on living with the condition.