Topic Resources
Erythromelalgia is a functional peripheral artery disease. Usually, the cause of erythromelalgia is unknown. In such cases, the disorder tends to start when people are in their 20s or older. A rare hereditary form of erythromelalgia starts at birth or during childhood.
Less commonly, the disorder is related to the use of some medications, such as nifedipine (a medication used to treat Less commonly, the disorder is related to the use of some medications, such as nifedipine (a medication used to treathigh blood pressure) or bromocriptine (a medication used to treat ) or bromocriptine (a medication used to treatParkinson disease and other disorders).
Erythromelalgia also occurs sometimes in people who have
Certain blood disorders (myeloproliferative disorders)
Spinal cord disorders
Systemic lupus erythematosus (lupus)
Erythromelalgia usually develops 2 to 3 years before another underlying disorder is diagnosed.
Symptoms include burning pain in the feet or hands, which feel hot and appear red in individuals with light skin, and darker than usual in individuals with dark skin. Attacks are usually triggered by environmental temperatures of over 84° F (over about 29° C). Symptoms may remain mild for years or may progress and become completely incapacitating.
Diagnosis of erythromelalgia is based on the symptoms and the increase in skin temperature. Tests, such as blood cell counts, are usually done to help identify an underlying disorder. Genetic testing can confirm a diagnosis of hereditary erythromelalgia in a person who has onset of symptoms in childhood. Affected people should receive genetic counseling because there is a 50% chance they will pass the disorder onto their children.
Treatment of Erythromelalgia
Avoiding heat
Relieving symptoms
Medications
Erythromelalgia treatment includes avoiding exposure to heat, resting, elevating the legs or arms, and applying cold packs to the legs or arms or immersing them in cold water. These measures sometimes relieve symptoms or prevent attacks.
If an underlying disease that causes erythromelalgia is identified, treating that disease may relieve symptoms. Depending on the underlying cause, aspirin may be prescribed.If an underlying disease that causes erythromelalgia is identified, treating that disease may relieve symptoms. Depending on the underlying cause, aspirin may be prescribed.
If no underlying disorder is identified, several topical or oral medications may relieve symptoms. These include topical treatments such as lidocaine, a combination of amitriptyline and ketamine, midodrine, capsaicin, and menthol as well as some oral medications such as mexiletine and carbamazepine.If no underlying disorder is identified, several topical or oral medications may relieve symptoms. These include topical treatments such as lidocaine, a combination of amitriptyline and ketamine, midodrine, capsaicin, and menthol as well as some oral medications such as mexiletine and carbamazepine.
Gabapentin, venlafaxine, or sertraline may also be prescribed as alternative options whether or not an underlying cause is found.Gabapentin, venlafaxine, or sertraline may also be prescribed as alternative options whether or not an underlying cause is found.
Drugs Mentioned In This Article
