What specialty is most appropriate to manage erythromelalgia?

From the Guidelines

Dermatologists or neurologists are most appropriate to manage erythromelalgia, as they can address both the skin manifestations and neuropathic pain components of the condition. The condition often requires a multidisciplinary approach, with treatment typically beginning with trigger avoidance (heat, exercise, alcohol) and cooling strategies 1. First-line medications include aspirin for vasodilation-related symptoms and gabapentin, pregabalin, or tricyclic antidepressants like amitriptyline (starting at 10-25mg nightly) for neuropathic pain 1. Topical treatments such as lidocaine 5% patches or compounded creams containing amitriptyline and ketamine may provide localized relief. For refractory cases, specialists might consider sodium channel blockers like mexiletine (150-300mg three times daily) or intravenous lidocaine under monitored conditions. Vascular specialists may be consulted for cases with significant vascular involvement, while pain management specialists can assist with complex pain control 1.

Some key points to consider in the management of erythromelalgia include:

• Evaluating for underlying causes or associations, and treating or managing these conditions if present 1
• Providing patient counseling and maintaining a lifestyle that is as normal as possible 1
• Cooling affected areas safely, such as with ice and water immersion of extremities for limited periods 1
• Considering referral to a comprehensive pain rehabilitation center for patients with severe, refractory, or disabling EM 1

The condition's pathophysiology involves small fiber neuropathy and vascular dysfunction, often requiring tailored treatment approaches based on whether the case is primary (idiopathic) or secondary to another condition 1. A multidisciplinary team approach is beneficial in the care of patients with EM, and may include dermatologists, neurologists, vascular specialists, and pain management specialists.

From the Research

Management of Erythromelalgia

The management of erythromelalgia involves a multidisciplinary approach, and the most appropriate specialty to manage the condition is not explicitly stated in the provided studies. However, based on the information provided, the following specialties may be involved in the management of erythromelalgia:

• Dermatology: Erythromelalgia is a condition characterized by skin symptoms such as redness, warmth, and burning pain, making dermatology a relevant specialty in its management 2, 3, 4.
• Hematology: Secondary erythromelalgia is associated with myeloproliferative disorders, which are typically managed by hematologists 5, 4.
• Neurology: Primary erythromelalgia is considered an autosomal dominant neuropathy, and some patients may experience small fiber neuropathy, making neurology a relevant specialty in its management 4.
• Pain management: Erythromelalgia is characterized by severe burning pain, and pain management specialists may be involved in its management, particularly in cases where procedural interventions are considered 6.
• Internal medicine: Erythromelalgia may be associated with various underlying conditions, such as rheumatic diseases or viral infections, making internal medicine a relevant specialty in its management 4.

Treatment Options

The treatment of erythromelalgia typically involves a combination of medications and procedural interventions, including:

• Aspirin: A useful treatment for erythromelalgia associated with myeloproliferative disorders 5, 4.
• Sodium channel blockers: Such as lidocaine, carbamazepine, and mexiletine, which may be used to treat primary erythromelalgia 4.
• Procedural interventions: Such as epidural infusion, sympathetic ganglion block, sympathectomy, pulsed radiofrequency, spinal cord stimulation, dorsal root ganglion stimulation, brain stimulation, transcranial magnetic stimulation, and botulinum toxin injections, which may be considered in cases where topical and systemic medications are ineffective 6.