Merck Manual

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Erythromelalgia

By

Koon K. Teo

, MBBCh, PhD, McMaster University

Medically Reviewed May 2021 | Modified Sep 2022
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Erythromelalgia is a rare syndrome in which small arteries (arterioles) of the skin dilate periodically, causing a burning pain, making the skin feel hot, and making the feet and, less often, the hands turn red.

Erythromelalgia is a functional peripheral arterial disease Overview of Functional Peripheral Arterial Disease Functional peripheral arterial disease is much less common than occlusive peripheral arterial disease. Normally, the arteries of the arms and legs widen (dilate) and narrow (constrict) in response... read more . 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.

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.

Treatment of Erythromelalgia

  • Avoiding heat

  • Relieving symptoms

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.

Drugs Mentioned In This Article

Generic Name Select Brand Names
Adalat, Adalat CC, Afeditab CR, Nifediac CC, Nifedical XL, Procardia, Procardia XL
Cycloset, Parlodel
Active-PAC with Gabapentin, Gabarone , Gralise, Horizant, Neurontin
Anacin Adult Low Strength, Aspergum, Aspir-Low, Aspirtab , Aspir-Trin , Bayer Advanced Aspirin, Bayer Aspirin, Bayer Aspirin Extra Strength, Bayer Aspirin Plus, Bayer Aspirin Regimen, Bayer Children's Aspirin, Bayer Extra Strength, Bayer Extra Strength Plus, Bayer Genuine Aspirin, Bayer Low Dose Aspirin Regimen, Bayer Womens Aspirin , BeneHealth Aspirin, Bufferin, Bufferin Extra Strength, Bufferin Low Dose, DURLAZA, Easprin , Ecotrin, Ecotrin Low Strength, Genacote, Halfprin, MiniPrin, St. Joseph Adult Low Strength, St. Joseph Aspirin, VAZALORE, Zero Order Release Aspirin, ZORprin
NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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