INFO - On erythromelalgia

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Merck Manuals

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Last updated Jan 2008

" Erythromelalgia "

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). 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.

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