What Is the Cause Of Erythromelalgia?

Erythromelalgia (EM) is a rare disease that makes various parts of the body swell and turn red, leading to severe pain, swelling, and discomfort. The feet and hands are the most common body parts affected by the health condition, which can have various causes, as highlighted below.

The good news is more is being learned about EM every day, and there are outstanding mental health professionals available online to help you deal with this condition.

Primary Erythromelalgia

This type of EM refers to genetic conditions that may cause the disorder, and it also refers to idiopathic conditions, meaning there is no known cause.

Primary EM happens more often in children and adolescents, but people of all ages can be affected. For younger people, sometimes the symptoms start during puberty.

This form of EM involves redness, pain, and warmth in the affected parts of the body; in some cases, the pain is debilitating. For example, pain and swelling in the hands can prevent you from eating, dressing, taking a bath, writing, etc.

Others have pain in their feet, so it prevents them from standing or walking when the pain persists.

Primary erythromelalgia attacks usually are worse when the weather is warmer and at night. It also can be brought on by sweating, exercise, or standing or sitting for too long.

Primary EM is understood to arise from a genetic condition that is often inherited. Unfortunately, the cause may be unknown; some patients have primary EM but have no family history of the problem.

Some families report that people in several generations had EM, so these cases may have something to do with autosomal dominant inheritance. But in others, the health problem may be caused by a genetic mutation in the sperm or egg cell, so it is not inherited.

Secondary Erythromelalgia

This type of EM is understood to stem from an underlying health condition. Some sources report that bone marrow problems, including thrombocythemia and polycythemia, are the most common causes.

However, secondary EM also may be related to the following health problems:

  • Gout
  • High blood pressure
  • Multiple sclerosis
  • Spinal cord conditions
  • Lead poisoning
  • Lupus
  • Rheumatoid arthritis

There is more evidence that secondary erythromelalgia may be caused in some people by large or small fiber neuropathies. Also, other evidence indicates that EM may happen as an adverse event related to taking certain drugs, such as nicardipine and nifedipine.

Still other evidence suggests that secondary EM may be caused by the unusual narrowing of capillaries in various parts of the body.

Erythromelalgia Diagnosis

A complete medical evaluation is needed to diagnose this condition potentially. Photographs of the areas affected by EM can be helpful to your healthcare professional.

For example, when you have an attack, have a loved one or friend take photos of the areas affected, such as your hands and feet. This can be vital to a correct diagnosis because the redness from EM is unique to the condition.

Your family and patient history also can be helpful for a diagnosis, and blood tests can rule out some health conditions. For instance, because EM can be an early indicator or thrombocythemia, lab tests that include red blood cell counts can help diagnose EM.

Some researchers say that the on-off nature of erythromelalgia can make it harder to diagnose. Again, that’s why doctors recommend taking pictures when you have a flare-up so your healthcare professional can see your symptoms as they occur.

EM can be a challenging health problem to diagnose and live with, but more is being learned about the condition every day. Fortunately, there are erythromelalgia support groups online that can help you manage the disorder and receive support.