Erythromelalgia Clinical Presentation

Updated: May 25, 2022
  • Author: Drew H Barnes, MD; Chief Editor: Emmanuel C Besa, MD  more...
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History

The classic description of erythromelalgia is a triad of redness, pain, and warmth in the extremities, brought on by warming or dependency and relieved by cooling (see the image below).

Cardinal symptoms of erythromelalgia. Cardinal symptoms of erythromelalgia.

Paroxysmal burning pain has also been reported as a presenting symptom of erythromelalgia. [31] Erythromelalgia has rarely been reported to involve parts other than the extremities (eg, ears, face, genitals). [32, 33, 34]

Symptomatic episodes may last minutes to days. They often begin with an itching sensation and later progress to more severe pain with a burning quality. Pain may be so intense that the patient cannot walk; some must even keep their feet immersed in ice water.

The lower extremities are affected more often than the upper extremities. The soles of feet and toes are most commonly involved. In rare cases, involvement may reach as high as the knees. Involvement is usually bilateral, though not necessarily symmetric. Warming the extremity or placing and maintaining the extremity in a dependent position can exacerbate symptoms; cooling and elevating the extremity can relieve symptoms.

Raynaud phenomenon has been reported to occur between episodes of erythromelalgia, but this may be coincidental.

In cases associated with a myeloproliferative disorder, erythromelalgia usually precedes the diagnosis of the myeloproliferative disorder by a median of 2.5 years. Rapid relief with aspirin is typical of this type of erythromelalgia and can be supportive diagnostically.

Given the association with myeloproliferative disorders, the clinician should inquire about other possible clinical manifestations of myeloproliferative disorders, such as:

  • Pruritus
  • Thrombotic episodes (eg, transient visual loss)
  • Constitutional symptoms
  • Abdominal discomfort 
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Physical Examination

Between episodes, examination findings may be normal.

The affected extremity becomes warm and tender during an episode and appears dusky, red, and sometimes mottled.

Peripheral pulses may be normal or bounding. Acrocyanosis may be observed; rarely, it progresses to distal digital necrosis. Ischemic ulcers may be observed and may become infected secondarily.

Checking for splenomegaly and lymphadenopathy should be part of a comprehensive physical examination in a patient with suspected erythromelalgia.

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Complications

Complications appear to be more frequent in patients who clearly have platelet-mediated disease.

Ulceration, necrosis, and gangrene of affected extremities are possible. Digital necrosis or skin ueration with secondary infection can lead to amputation. At least one patient had near-fatal hypothermia related to constant cooling required to control symptoms.

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