Erythromelalgia

What is erythromelalgia?

Erythromelalgia (ur-i-thruh-muh-lal-jah) is a rare condition in which excess blood flow causes periodic symptom flares. It’s a chronic condition, meaning that it stays with you for life. Erythromelalgia symptoms, which often include burning pain, worsen over time.

Erythromelalgia typically affects your feet but can also occur on your:

• Arms.
• Face.
• Hands.
• Legs.

Are there different types of erythromelalgia?

There are several types, including:

Primary erythromelalgia is isolated and not due to an underlying disease.

• Idiopathic erythromelalgia is the most common type and occurs for unknown reasons.
• Inherited erythromelalgia is due to gene mutations. The mutations, which affect pain signals, can be passed down in families.

Secondary erythromelalgia occurs when there is an underlying blood, neurological or immunologic condition. These include:

• Autoimmune diseases, such as lupus and multiple sclerosis.
• Essential thrombocythemia, a blood disorder that causes your body to produce too many platelets.
• Polycythemia vera.
• Raynaud's phenomenon.
• Thrombocytopenia.
• Neuropathy.

What are erythromelalgia symptoms?

The three primary erythromelalgia symptoms are:

1. Increase in skin temperature.
2. Pain that ranges from mild tingling to severe burning.
3. Skin redness.

Symptoms may also include:

• Itching.
• Skin that’s cold to the touch between flares.
• Sweating in an area more than you usually would.
• Swelling.
• Tender, blotchy skin.
• Tingling sensation.

Symptoms may occur on one or both sides of your body. They can range from mild to intense. Some people experience their first flare in childhood, while others aren’t affected until adulthood.

What causes symptom flares?

Flares usually occur when you experience a sudden increase in body temperature. This may happen when you are:

• Consuming caffeinated beverages.
• Dehydrated.
• Drinking alcohol.
• Eating spicy food.
• Exercising.
• Feeling stressed.
• In a warm room or environment.

How is erythromelalgia diagnosed?

There’s no specific test for diagnosing erythromelalgia. Your healthcare provider reviews your symptoms and examines any active flare-ups or photos of flare-ups. Multiple tests may be necessary to confirm your symptoms and rule out other medical issues. These include:

• Blood tests.
• Genetic testing to check for mutations responsible for the disease.
• Thermography, which uses a special camera to pinpoint differences in skin temperature.
• X-ray or other imaging studies.

Can an ice bath help me?

It may be tempting to soak the affected area in ice water, but ice baths can make erythromelalgia symptoms worse. It can also put you at risk for skin issues like nonhealing wounds and necrosis that are difficult to treat.

What does erythromelalgia treatment involve?

The treatment that’s right for you depends on the cause. For secondary erythromelalgia, treating the underlying issue may relieve your symptoms. Treatment for primary erythromelalgia includes medications.

What medications might I need?

Erythromelalgia treatment often includes drugs you take by mouth. Many of these drugs are for other medical conditions. But they can be effective in relieving erythromelalgia symptoms.

They include:

• Anticonvulsants, such as gabapentin or carbamazepine.
• Antidepressants, like amitriptyline or venlafaxine.
• Antihistamines, such as cetirizine or diphenhydramine.
• Aspirin (for adults only).
• Blood pressure medicines to increase or decrease blood flow.
• Prescription painkillers.

Are there other treatment options?

Erythromelalgia treatment may also include:

• Lidocaine infusions, a pain medication you receive through a vein in your arm.
• Nutritional supplements, such as magnesium and alpha-lipoic acid.
• Pain management therapies, including nerve blocks.
• Topical medications, such as creams you apply to your skin.

Is there anything else I can do to get relief?

Nonmedical therapies include:

• Elevating the affected area.
• Resting on a cool surface, such as a tile floor.
• Using a fan.
• Wrapping cool first-aid gel packs in a thin towel and placing them on your skin.

Can erythromelalgia be prevented?

There isn’t much you can do to prevent erythromelalgia. If you have a family history of the disease, genetic testing can uncover the likelihood of passing it on to a fetus.

What is the prognosis for people with erythromelalgia?

Symptom flares can affect your quality of life, but they are rarely fatal. Most people live well into older adulthood. They have jobs, travel, enjoy hobbies and more. Sometimes erythromelalgia treatment is not successful, making it difficult to go about your daily routine.

How is daily life with erythromelalgia?

People who have the disease say that symptom flares can feel like:

• A binding sensation, as if your skin is too tight.
• Being on fire or scalded by hot water.
• Razor blades cutting your skin.
• Severe sunburn.

What can I do to prevent symptom flares and stay comfortable?

There are many steps you can take to feel your best and ease discomfort. These include:

• Avoiding food and beverage triggers: Pay careful attention to what you eat and drink so that you don’t consume items like caffeine or spicy foods that may raise your temperature.
• Exercising: Choose gentle activities that get you moving without significantly raising your temperature. These include swimming, yoga and Tai Chi.
• Staying in cool environments: If you will be outdoors, try to do so in the morning when temperatures are cooler.
• Bathing carefully: Choose showers instead of baths to avoid skin injuries. You can also keep your body cool by opening a window and using lukewarm water.
• Planning ahead: Since symptom flares tend to occur at night, take care of essential tasks earlier in the day.
• Getting organized before sleeping: Keep a fan or portable air conditioning unit near your bed. And use the lightest possible bed sheets or no top sheets at all.

How can I take care of my mental health?

Many people feel isolated and not understood by their family members or healthcare providers. Online erythromelalgia support groups enable you to connect with people who are going through similar challenges. Learning from the experiences of others can help you feel less alone.

A note from Cleveland Clinic

Erythromelalgia is a rare condition that causes episodes of pain and burning skin. There are many treatment options, and they typically include medications. Sometimes medications aren’t enough to quiet symptom flares, making it difficult to go about your daily routine. Even with these setbacks, many people live long, rewarding lives.