From the Guidelines
Erythromelalgia should be managed with a multimodal approach, including lifestyle modifications and pharmacologic treatments, with aspirin being a first-line systemic treatment option, given its low cost and relatively low adverse effect profile, as evidenced by a recent review of medical management options 1.
Diagnosis and Pathophysiology
Erythromelalgia is a rare neurovascular disorder characterized by episodes of burning pain, redness, and increased skin temperature primarily affecting the extremities, especially the feet and hands. The condition stems from dysfunction in peripheral sensory neurons and small fiber neuropathy, causing abnormal vasodilation and pain. Symptoms often worsen with heat because temperature-sensitive ion channels in affected nerves become hyperactive, triggering pain and vascular changes.
Treatment Approach
Treatment typically involves avoiding triggers such as heat, strenuous exercise, and alcohol, while cooling affected areas with cold water or ice can provide temporary relief.
- First-line medications include topical agents like lidocaine patches or amitriptyline creams.
- Oral medications such as gabapentin (starting at 300mg daily, gradually increasing to 900-3600mg daily in divided doses), pregabalin (150-600mg daily in divided doses), or amitriptyline (10-75mg at bedtime) may also be effective.
- Aspirin (325mg daily) is a recommended first-line systemic treatment option, particularly in patients with secondary EM due to underlying myeloproliferative disease, as it reduces platelet aggregation and improves circulation 1.
- For more severe cases, calcium channel blockers like amlodipine (5-10mg daily) or verapamil may help by improving blood flow.
- Refractory cases might require consultation with pain specialists for nerve blocks or spinal cord stimulation.
Quality of Life and Outcomes
Patients with erythromelalgia have lower quality of life outcomes, increased morbidity and mortality, and increased risk for suicide compared with the general population 1. Effective treatment resulting in symptomatic relief is essential to avoid severe complications and to improve outcomes. Therefore, a prompt and comprehensive treatment approach is crucial to manage erythromelalgia and improve patient outcomes, with aspirin being a viable first-line option 1.
From the Research
Definition and Characteristics of Erythromelalgia
- Erythromelalgia is a rare condition characterized by intense, burning pain and redness primarily of the feet, and sometimes the hands 2.
- It is a microvascular disease with unknown etiology, and most cases are idiopathic (primary EM), while others occur secondary to medical conditions such as autoimmune diseases, neurological or hematological disorders 2.
- Symptoms are episodic and can result in severe disability, with triggers such as exposure to warmth, pressure, or exercise 2.
Diagnosis and Treatment
- There are no diagnostic tests for erythromelalgia, and diagnosis is based on history, physical examination during symptomatic episode, and the exclusion of other probable causes for the syndrome 2.
- Treatment options include topical and systemic medications, as well as procedural interventions such as epidural infusion, sympathetic ganglion block, and spinal cord stimulation 3.
- A multidisciplinary approach to management may be needed for patients with erythromelalgia, and patient education is crucial to prevent complications such as acral necrosis, infection, and amputation 4.
- Gabapentin has been reported to be effective in treating erythromelalgia in some cases 5.
Forms and Prognosis
- Primary erythromelalgia is considered to be a genetically determined neuropathic disorder affecting neuronal sodium channels, while secondary forms may be associated with myeloproliferative disorders, connective tissue disease, cancer, infections, and poisoning 6.
- Prognosis mainly depends on the underlying condition and the ability of the patients and their relatives to cope with the disease 6.
- Genetic testing is recommended, especially in premature cases and in cases of family clustering, to differentiate between primary and secondary forms of erythromelalgia 6.