Erythromelalgia Clinical Presentation
- Author: Antoine N Saliba, MD; Chief Editor: Emmanuel C Besa, MD more...
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).
Paroxysmal burning pain has been also reported to be a presenting symptom of erythromelalgia. Erythromelalgia has also been reported to involve parts other than the extremities (eg, auricular erythromelalgia).
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. Involvement as high as the knees is observed but is rare. 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 a possible history of pruritus, thrombotic episodes (eg, transient visual loss), constitutional symptoms, and abdominal discomfort that could accompany myeloproliferative disorders.
Between episodes, examination findings may be normal. During an episode, the affected extremity becomes warm, 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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