Erythromelalgia: Causes, Symptoms, Diagnosis, Treatment, and Prevention
The condition erythromelalgia is characterized by pain, redness, warmth, and swelling in various parts of the body, particularly in the hands and feet. Although it affects people of any age, gender, or ethnicity, it is more common in women and older adults. A person's symptoms may vary in severity and frequency, and they may be triggered by a variety of factors, including heat, stress, exercise, alcohol, or spicy foods.
Causes of Erythromelalgia
The exact cause of erythromelalgia is not fully understood, but it is believed to involve abnormal blood flow and nerve function in the affected areas. Some possible causes or risk factors include:
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Genetic mutations that affect the sodium channels in the nerves, which regulate pain and temperature sensations. These mutations can be inherited or acquired.
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Blood disorders that increase the number or activity of platelets, which are cells that help with blood clotting. These disorders include essential thrombocythemia and polycythemia vera.
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Autoimmune diseases that cause inflammation and damage to the tissues, such as lupus, rheumatoid arthritis, or multiple sclerosis.
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Neurological diseases that affect the nerves or brain, such as neuropathy, stroke, or Parkinson’s disease.
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Medications that dilate the blood vessels, such as calcium channel blockers, nifedipine, or nicardipine.
Symptoms of Erythromelalgia
The main symptoms of erythromelalgia are:
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Pain that ranges from mild tingling to severe burning in the affected areas.
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Redness and increased skin temperature in the affected areas, which can be measured by a thermometer or felt by touch.
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Swelling and tenderness in the affected areas, which can make them look puffy or shiny.
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Itching and sweating in the affected areas, which can worsen the discomfort.
Most commonly, the symptoms occur on both sides of the body, but they can also affect just one side or different parts of the body. Hands and feet are most commonly affected, but the face, ears, legs, arms, or trunk are also susceptible. The symptoms can last from minutes to hours, and they can come and go unpredictably. They tend to worsen at night and in warm environments, and they can interfere with daily activities and sleep quality.
Diagnosis of Erythromelalgia
Erthromelalgia cannot be diagnosed by a specific test, but it is diagnosed by examining the patient's clinical features and medical history. In addition, the doctor may conduct some tests to rule out other conditions that can cause similar symptoms, such as infections, allergies, or vascular diseases.
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Blood tests to check the levels of platelets, red blood cells, and inflammatory markers.
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Skin biopsy to examine the tissue under a microscope and look for signs of inflammation or nerve damage.
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Thermography to measure the skin temperature and blood flow in the affected areas using infrared cameras.
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Nerve conduction studies to assess the function and speed of the nerves in the affected areas using electrical impulses.
Treatment of Erythromelalgia
There is no cure for erythromelalgia, but the treatment aims to reduce the frequency and severity of the symptoms and improve the quality of life of the patient. The treatment options include:
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Change your lifestyle, such as avoiding triggers, wearing loose and breathable clothing and footwear, elevating the affected areas, applying cool compresses or ice packs, and using fans and air conditioners.
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There are medications that can lower the platelet count and improve blood flow, as well as gabapentin, pregabalin, and carbamazepine, which modulate nerve signals and reduce pain. In some cases, lidocaine, capsaicin, or opioids may also be used, but their side effects and risks are greater.
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Nerve blocks, which use local anesthetics to temporarily numb nerves in the affected areas, or sympathectomy, which uses surgery or radiofrequency ablation to permanently cut or destroy nerves controlling blood vessels in those areas. The procedures are typically reserved for severe or refractory cases, and may result in complications or adverse effects.
Prevention of Erythromelalgia
There is no known way to prevent erythromelalgia, but some measures that may help reduce the risk or delay the onset of the condition include:
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Maintaining a healthy weight and blood pressure, which can improve the blood circulation and reduce the stress on the blood vessels.
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Avoiding smoking and excessive alcohol consumption, which can damage the blood vessels and nerves and impair the blood flow.
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Managing any underlying conditions that may contribute to erythromelalgia, such as blood disorders, autoimmune diseases, or neurological diseases, by following the doctor’s advice and taking the prescribed medications.
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Seeking medical attention promptly if any symptoms of erythromelalgia occur, especially if they are severe or persistent, to get an accurate diagnosis and appropriate treatment.
