Erythromelalgia Clinical Presentation

Updated: Sep 17, 2018
  • Author: Antoine N Saliba, 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 been also reported to be a presenting symptom of erythromelalgia. [27] Erythromelalgia has also been reported to involve parts other than the extremities (eg, ears, face, genitals). [28, 29, 30]

Symptomatic episodes may last minutes to days. They often begin with an itching sensation, progressing to a 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 diagnosis of the myeloproliferative disorder by a median of 2.5 years. Dramatic relief with aspirin is typical of this type of erythromelalgia and can be used as an aid to diagnosis.

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

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

Between episodes, examination findings may be normal. During an episode, the affected extremity becomes warm and tender, and appears dusky, red, and sometimes mottled.

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

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

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