From the Guidelines
Erythromelalgia diagnosis and treatment should prioritize a comprehensive approach, starting with trigger avoidance, patient counseling, and evaluation for underlying causes, followed by first-line therapy with topical agents or aspirin 325mg daily, as recommended by the most recent guidelines 1. The diagnosis of erythromelalgia is based on clinical symptoms, including episodic burning pain, redness, and increased temperature in the extremities, typically triggered by heat exposure or exercise. Diagnosis requires excluding other conditions through a thorough medical history, physical examination, and sometimes specialized tests like skin biopsies or nerve conduction studies.
Treatment Approach
Treatment begins with trigger avoidance, such as keeping affected areas cool and elevated, and evaluating for underlying causes or associations, which should be treated or managed accordingly 1.
- First-line therapy includes topical agents, each for a trial of 4 weeks, or aspirin 325mg orally daily for a trial of 1 month, as it is probably effective through its mechanism of inhibiting both prostaglandin synthesis and platelet aggregation 1.
- For systemic treatment, oral medications such as gabapentin, pregabalin, or amitriptyline may be considered, although the evidence for these is not as strong as for aspirin in certain cases 1.
Medication Considerations
- Aspirin is especially effective in patients who have secondary erythromelalgia due to underlying myeloproliferative disease, such as essential thrombocytosis and polycythemia vera 1.
- Mexiletine, a nonselective sodium channel antagonist, is a safer oral alternative to intravenous lidocaine infusions and may be considered for refractory cases 1.
- Serotonin-norepinephrine reuptake inhibitors, such as venlafaxine, have been used with variable success and may be considered in certain cases 1.
Specialist Referral
Pain management specialists might recommend nerve blocks or spinal cord stimulation for severe cases, and referral to a pain rehabilitation center may be necessary for third-line therapy 1. Treatment is typically long-term and requires monitoring for medication side effects and efficacy, with dose adjustments as needed. The condition stems from small fiber neuropathy or vasculopathy causing abnormal pain sensation and blood flow regulation, which explains why medications targeting nerve pain and vascular function are effective.
From the Research
Diagnosis of Erythromelalgia
- Erythromelalgia is a rare intermittent vascular acrosyndrome characterized by the combination of recurrent burning pain, warmth, and redness of the extremities 2
- It can be classified as primary or idiopathic if there is no accompanying disease process, or secondary if associated with myeloproliferative syndrome-related thrombocythemia, or other underlying conditions 3
- Diagnosis is based on clinical presentation, including a burning sensation with erythema of the involved extremity, intensified by heat or lowering of the extremity, and relieved by cold or elevation 3
Treatment of Erythromelalgia
- Treatment of primary erythromelalgia is difficult and individualized, with sodium channel blockers such as lidocaine, carbamazepine, and mexiletine being used 2
- Aspirin is a useful treatment for erythromelalgia associated with myeloproliferative disorders 2, 3
- Procedural interventions, including epidural infusion, sympathetic ganglion block, sympathectomy, pulsed radiofrequency, spinal cord stimulation, dorsal root ganglion stimulation, brain stimulation, transcranial magnetic stimulation, and botulinum toxin injections, have been used with varying degrees of success 4
- A multidisciplinary approach to management is often needed, including patient education, avoidance of triggers, control of secondary and underlying factors, and use of medications to control symptoms 5, 6
- The goal of management is pain control, reduction of flare frequency, and prevention of complications, with patient education being crucial to prevent unsafe self-administered cooling techniques 6