Erythromelalgia Treatment & Management

Updated: Jul 31, 2017
  • Author: Antoine N Saliba, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment

Approach Considerations

Evaluation and treatment can be conducted in an outpatient setting. Treatment is primarily medical and supportive. Local measures, such as cooling or elevating the extremity, may relieve symptoms. Avoid excessive warming or dependency of the extremity. The environment should be modified so that it is not too hot.

In patients with myeloproliferative disorders, chemotherapy to reduce the platelet count often alleviates symptoms, but it is not universally effective. Some patients with polycythemia vera have responded to phlebotomy. A hematologist may be consulted if an associated myeloproliferative disorder is present.

Surgery probably has no role in the management of erythromelalgia except to treat the rare complication of gangrene. Surgical sympathectomy has been attempted, with variable results.

No specific dietary restrictions are necessary. Vigorous exercise may induce an acute episode and should therefore be avoided.

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Pharmacologic Therapy

For primary erythromelalgia, the best therapy is unknown. Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, gabapentin, and typhoid vaccine appear in the literature, as well as case series with high-dose magnesium, [28] intravenous bupivacaine, the lidocaine patch, [29] andd lidocaine plus mexiletine. [30] A study in 12 patients reported benefit with the alpha1-agonist midodrine, 0.2%, compounded in a moisturizing skin cream and applied topically three times a day during symptoms. [31]

Medications that affect voltage-gated sodium channels (eg, lidocaine and mexiletine) show promise. [32, 33, 34] Prostacyclin may provide some benefit [35] ; some patients achieve relief with gabapentin or high-dose magnesium. [28] The author knows of no randomized trial of therapy for erythromelalgia. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms.

Poterucha et al treated 36 patients with compounded topical amitriptyline-ketamine; 75% of those patients reported a reduction in pain. [36]

A Mayo Clinic study in 31 patients found that response to treatment with corticosteroids was more likely to occur in patients who reported a precipitant for their erythromelalgia (eg, surgery, trauma, or infection) and in those who had a subacute temporal profile to disease zenith (<21 days). In addition, patients who responded to steroids started treatment sooner—at 3 (3-12) versus 24 (17-45) months (P = 0.003). [37]

In cases that are associated with other disorders, treating the original disease might improve symptoms. One case report described a patient with seronegative polyarthritis who developed erythromelalgia and was successfully treated with intravenous immunoglobulins. [38]

For erythromelalgia related to thrombocytosis, aspirin is usually the treatment of choice. Other nonsteroidal anti-inflammatory drugs (NSAIDs) provide relief of short duration. Anagrelide may be an alternative. Other platelet-inhibiting agents (eg, ticlopidine and dipyridamole) have no effect.

Monitor for complications, response to treatment, and development of a myeloproliferative disorder because erythromelalgia often precedes the clinical appearance of polycythemia vera or essential thrombocythemia.

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