From the Guidelines
Erythromelalgia is a rare neurovascular disorder characterized by episodes of burning pain, redness, and swelling primarily affecting the extremities, especially the feet and hands, and its management should start with aspirin as the first-line systemic treatment, given its low cost and relatively low adverse effect profile, at a daily dose of 325mg for at least 1 month, as supported by recent evidence 1. The condition is triggered by warmth and relieved by cooling, resulting from abnormal blood flow regulation, and can be either primary or secondary to underlying conditions like blood disorders, autoimmune diseases, or medication side effects. Some key characteristics of erythromelalgia include:
- Intense burning pain
- Redness
- Warmth
- Swelling
- Symptoms worsen with heat, exercise, or dependency of limbs Management of erythromelalgia focuses on avoiding triggers, cooling affected areas, and medications targeting the pain. Aspirin is especially effective in patients who have secondary erythromelalgia due to underlying myeloproliferative disease, such as essential thrombocytosis and polycythemia vera, with dramatic improvement reported to occur within days of aspirin initiation, as noted in a case series of 57 patients 1. The antiplatelet effects of aspirin make it particularly useful in this subset of patients. Other treatment options may include topical agents, oral medications, and sodium channel blockers, but aspirin should be explored as the initial therapy due to its overall safety, accessibility, and effectiveness, particularly in patients with myeloproliferative disease 1.
From the Research
Overview of Erythromelalgia
Erythromelalgia is a rare neurovascular pain syndrome characterized by a triad of symptoms, including:
- Redness
- Increased temperature
- Burning pain primarily in the extremities, such as the feet and hands 2, 3, 4, 5 The condition can be classified into primary and secondary forms, with secondary erythromelalgia often associated with myeloproliferative diseases, such as essential thrombocythemia and polycythemia vera 2, 3, 5, 6.
Clinical Characteristics
The symptoms of erythromelalgia are typically provoked by:
- Environmental heat
- Exercise
- Dependency of the affected extremity And are relieved by:
- Exposure to cold
- Elevation of the extremity 2, 5 In some cases, erythromelalgia may be a precursor to an underlying myeloproliferative disease, with symptoms often preceding the onset of the disease by several years 2, 5.
Treatment
Treatment of erythromelalgia varies depending on the underlying cause, but may include:
- Aspirin therapy, which has been shown to be effective in relieving symptoms in patients with secondary erythromelalgia associated with myeloproliferative diseases 2, 3, 5, 6
- Sodium channel blockers, such as lidocaine, carbamazepine, and mexiletine, which may be used to treat primary erythromelalgia 4