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What Is Erythromelalgia?

Medically Reviewed by Poonam Sachdev on August 25, 2022

Erythromelalgia is a rare disorder that causes intermittent sensations of burning and redness. It primarily occurs on the feet. In rare cases, it can also affect the hands, arms, legs, face, and ears. 

Erythromelalgia can occur at any age. Some patients have been diagnosed with it from childhood, but others have only received a diagnosis as an adult. 

Erythromelalgia Causes

There are a few types and subtypes of erythromelalgia, each with its own causes. These types include: 

Primary erythromelalgia 

Primary erythromelalgia occurs in isolation without any underlying diseases. There are two primary types: 

  • Idiopathic: The most common form of erythromelalgia. It occurs with no known cause.
  • Inherited: A defective gene is passed down from parent to child.

Secondary erythromelalgia 

Secondary erythromelalgia results from an underlying condition relating to neurological, blood, or immunological disorders. These conditions include: 

  • Lupus, multiple sclerosis, and other autoimmune disorders 
  • Essential thrombocythemia – a disorder of the blood that makes your body develop too many platelets 
  • Polycythemia vera – a type of blood cancer 
  • Raynaud’s phenomenon – a condition that affects certain body parts, making them feel numb and turn different colors 
  • Thrombocytopenia – a condition in which the platelet count in your blood is lower than it should be
  • Neuropathy – damaged or defective nerves resulting in muscle weakness, numbness, tingling, and pain in affected areas

Erythromelalgia Symptoms

The main symptoms of erythromelalgia include an elevated skin temperature, pain that can be mild to severe in nature, and reddening skin. 

Other symptoms may be present as well. These symptoms include: 

  • Itching
  • Skin that seems cold in between flareups 
  • Excessive sweating
  • Swelling
  • Blotchy skin that is tender 
  • Feelings of tingling

Symptoms may present themselves on one side of your body or on both sides and can range in severity from mild to severe. 

Flareup Causes

Erythromelalgia occurs in episodes, and certain factors can cause an episode, or a flareup, to occur. These flareups are associated with an elevated body temperature. Factors that contribute to an elevated body temperature include: 

  • Drinking caffeine 
  • Becoming dehydrated
  • Alcohol consumption
  • Consuming spicy foods
  • High-intensity workouts
  • Stressful situations
  • A warm environment 

Diagnosing Erythromelalgia

There are no tests or diagnosing tools available that are specific to erythromelalgia. Instead, your doctor will review your symptoms and observe any active flareups. If no active flareups are present, then photographic evidence may be needed. Other tests may be conducted to ensure that no other medical conditions are the culprit of your symptoms. These tests include: 

  • Blood tests
  • Genetic testing 
  • Thermography
  • Imaging tests such as x-rays 

 Unfortunately, erythromelalgia can be difficult to diagnose. Its episodic nature often leads to delays in receiving a proper diagnosis. Since symptoms may occur later in the day, some doctors recommend taking pictures of the affected areas during flareups after operating hours. Alternatively, patients can request an appointment later in the day, if possible, to be evaluated by their doctor. 

Sometimes, doctors may require patients to exercise or to be immersed in hot water for up to 30 minutes to provoke a flareup. 

Erythromelalgia Prognosis

Erythromelalgia is seldom fatal. Still, this disorder can affect the quality of your life during flareups. Most patients live a long and full life and are able to work, travel, and enjoy hobbies. 

Sometimes, though, erythromelalgia treatment is unsuccessful, and the illness can make it difficult to carry out your daily activities. 

Erythromelalgia Treatment

There are many treatments for erythromelalgia, but they are dependent upon the cause. 

For those with secondary erythromelalgia, it may be helpful to treat the underlying disease causing erythromelalgia. 

For patients with primary erythromelalgia, medication is usually prescribed. These medications are usually oral and can be successful in relieving symptoms associated with erythromelalgia. 

These medications include: 

  • Gabapentin, carbamazepine, and other anticonvulsants 
  • Amitriptyline, venlafaxine, and other antidepressants 
  • Cetirizine, diphenhydramine, and other antihistamines 
  • Aspirin 
  • Certain blood pressure medications 
  • Prescription painkillers 

Other treatment methods include: 

  • Lidocaine injections received via a vein located in your arm
  • Magnesium, alpha-lipoic acid, and other nutritional supplements 
  • Pain management therapies
  • Tropical medications in the form of creams 

There are also some nonmedical treatment options available that you can practice at home. These include: 

  • Elevating the affected area
  • Allowing the affected area to rest in a cool area
  • Using a fan to cool yourself down 
  • Wrapping first-aid gel packs in a towel and applying them to your affected skin

Some patients may want to soak painful and red areas in ice water or to press an icy compress against the affected area. This can, however, cause erythromelalgia symptoms to worsen. Additionally, exposing your skin to icy conditions may put you at risk for certain skin issues such as nonhealing wounds and necrosis. 

Erythromelalgia Prevention

Erythromelalgia is not a preventable disease. Still, genetic testing can be done on those with a family history of erythromelalgia to discover whether or not there is a chance of your unborn child ending up with the condition.

How to Prevent Flareups and Manage Symptoms

While erythromelalgia is not wholly preventable, there are ways to prevent flareups from occurring as frequently and to be more comfortable when they do occur. 

If you wish to prevent flareups, consider the following: 

  • Avoid certain food and beverages: Caffeinated beverages and spicy foods can increase the temperature of your body, thus triggering an episode of erythromelalgia. Avoiding these drinks and foods can help prevent these episodes from occurring and prevent the symptoms from worsening. 
  • Adjust your exercises: Intense workouts can cause an increase in body temperature as well. If you plan on exercising, choose gentle activities such as swimming and yoga. 
  • Stay in cool conditions: It’s important when dealing with erythromelalgia that you stay in the coolest conditions possible. If you plan on participating in outdoor activities, aim to do so in the morning hours before the temperature rises. 
  • Be careful when bathing: To avoid skin wounds, choose showers over baths. Also, use lukewarm water and open a window to help you stay cool.
  • Be prepared: Flareups occur most commonly in the evening and nighttime hours and can affect your nightly chores and sleep schedule. Instead of waiting until late in the day to do important tasks, complete them in the morning and early afternoon. Additionally, you may want to squeeze in a nap, if possible, in the daytime hours.
  • Practice organization before sleeping: Organize the essentials prior to bedtime. Keeping a fan or portable air conditioner next to your bed can help you stay cool. You can also use the lightest bed sheets possible. Alternatively, you can also choose to use no top sheets at all to alleviate symptoms.

It’s also important to take care of your mental health when dealing with erythromelalgia. Because it’s a rare disorder, it’s hard for most people to understand what you’re going through. You may end up feeling isolated as a result. To help you not feel so alone, you can try to reach out to online erythromelalgia support groups to share your story and hear the stories of others.

Show Sources

SOURCES: 

NHS: “Erythromelalgia.”

Cleveland Clinic: “Erythromelalgia.”

Rare Diseases: “Erythromelalgia.”

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