Erythromelalgia is a functional peripheral arterial 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 drugs, such as nifedipine (a drug used to treat high blood pressure) or bromocriptine (a drug used to treat Parkinson disease and other disorders).
Erythromelalgia also occurs in people who have
Erythromelalgia usually develops 2 to 3 years before the disease that causes it is diagnosed.
Symptoms include burning pain in the feet or hands, which feel hot and appear red. 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 a cause. 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.
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. If no causative disease disorder is identified, gabapentin may relieve symptoms. Aspirin may be helpful when a myeloproliferative disorder is the underlying cause.