Erythromelalgia is a rare type of blood vessel disease whose symptoms include flare-ups of burning pain in the hands and feet, and less commonly in the arms, legs, and face.

The disease can occur alongside systemic scleroderma.

Erythromelalgia and scleroderma

Erythromelalgia is classed as primary when the cause is either genetic or unknown and  secondary when it is caused by another disease. Secondary erythromelalgia is usually associated with autoimmune conditions, which occur when the immune system mistakenly attacks healthy tissue. These conditions include systemic scleroderma.

Exactly how scleroderma causes erythromelalgia is unknown, but it is believed to be associated with too much blood flowing toward the hands and feet. This could be due to abnormal narrowing or widening of blood vessels.

Erythromelalgia is sometimes considered the opposite of Raynaud’s phenomenon, a common condition associated with scleroderma that causes blood to be directed away from the hands and feet, resulting in painful cold sensations. There are recorded cases of both erythromelalgia and Raynaud’s phenomenon being diagnosed in a single patient, as well as alongside scleroderma.

Symptoms of erythromelalgia

Erythromelalgia is characterized by periods where the affected area becomes red and hot to the touch. This is usually accompanied by pain ranging from mild discomfort and tingling to a severe burning sensation.

The affected area can also swell, and a flare-up can change the amount of sweat being produced there. Outside of the affected area the skin can appear bluish and be cold to the touch.

Diagnosis of erythromelalgia

There is no specific test to diagnose erythromelalgia. Examining the medical history of the patient and their family can help doctors identify conditions commonly associated with erythromelalgia, supporting the diagnosis.

In trying to obtain a diagnosis, a doctor may also ask that a patient take pictures during a flare-up or schedule an examination at a time of day when flare-ups often occur. For many patients, this is late in the day.

Treatment of erythromelalgia

There is no cure for erythromelalgia, but some therapies can alleviate symptoms. Identifying flare-up triggers, and making lifestyle changes to prevent them from occurring, can reduce the chance of a flare-up. Examples include keeping cool while exercising, reducing water temperature while bathing, and avoiding spicy food.

Some symptom relief can be achieved by cooling the affected area — with fans or cool gel packs, for example. Patients should avoid applying ice because its repeated use can lead to permanent skin and nerve damage. If a patient also has Raynaud’s, ice treatment can lead to a Raynaud’s attack.

A range of therapies can reduce the pain associated with erythromelalgia, but not all medications work for every patient. Examples include:

  • Lidoderm (lidocaine patches)
  • Aspirin
  • Nerve damage therapies. They include anti-epilepsy drugs such as Tegretol (carbamazepine) and gabapentin, and antidepressants such as Elavil (amitriptyline).

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