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Erythromelalgia
is distressing paroxysmal vasodilation of small arteries in the
feet and hands and, less commonly, in the face, ears, or knees;
it causes burning pain, increased skin temperature, and redness.
This rare disorder may be primary (cause unknown) or secondary to myeloproliferative disorders (eg, polycythemia vera, thrombocythemia), hypertension, venous insufficiency, diabetes mellitus, SLE, RA, lichen sclerosus, gout, spinal cord disorders, or multiple sclerosis. Less commonly, the disorder is related to the use of some drugs (eg, nifedipine , bromocriptine ). A rare hereditary form of erythromelalgia starts at birth or during childhood.
Burning pain, heat, and redness in the feet or hands last a few minutes to several hours. In most patients, symptoms are triggered by warmth (temperatures of 29 to 32° C) and are typically relieved by immersion in ice water. Trophic changes do not occur. Symptoms may remain mild for years or become severe enough to cause total disability. Generalized vasomotor dysfunction is common, and Raynaud's syndrome may occur.
Diagnosis is clinical. Testing is done to detect causes. Because erythromelalgia may precede a myeloproliferative disorder by several years, repeated blood counts may be indicated. Differential diagnosis includes posttraumatic reflex dystrophies, shoulder-hand syndrome, peripheral neuropathy, causalgia, Fabry's disease, and bacterial cellulitis.
Treatment is warmth avoidance, rest, elevation of the extremity, and application of cold. For primary erythromelalgia, gabapentin may be of benefit. For secondary erythromelalgia, the underlying disorder is treated; aspirin may be helpful when a myeloproliferative disorder is involved.
Last full review/revision January 2008 by John W. Hallett, Jr., MD
Content last modified January 2008
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