Erythromelalgia is classified as either primary or secondary. Primary Erythromelalgia (PE) is caused by a specific genetic mutation and results in affected patients developing an autoimmune disorder. Secondary erythromelalgia is often associated with myeloproliferative disorders. In some cases, secondary erythromelalgia occurs in neoplastic diseases and autoimmune neuropathies. Erythromelalgia is characterized by the combination of recurrent burning pain, warmth, and redness of the extremities. Burning severe pain is the most predominant symptom, and it can be disabling. The pain is usually caused and precipitated by warmth and physical activities. Bouts of pain typically start with an itch-like feeling and then progresses to a severe burning sensation with the duration ranging from several minutes to hours or even days. Pain symptoms are worse in summer and at night and are usually provoked and exacerbated by heat, ambulation, physical exercise, sitting, leg dependence, and coverage of extremities. Cooling and elevation are the most effective ways to relieve pain symptoms. Patients often immerse affected limbs in ice water, uncover their feet during sleep or walk barefoot in winter. Affected extremities can develop ulceration and gangrene which are not directly attributable to PE but are results of excessive exposure to low temperature in an attempt to relieve pain.
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