Current pain management strategies for patients with erythromelalgia: a critical review

Abstract

Erythromelalgia (EM) is a rare disorder characterized by erythematous, warm, painful extremities, which is often precipitated by cold conditions. The pathophysiology of EM is incompletely understood. Recent investigations have identified sodium channelopathy as a genetic cause for this pain condition, classified as primary inherited EM. Other subtypes are idiopathic EM and secondary EM. The management of pain in EM is challenging as no single therapy has been found to be effective. There is varying response to pharmacotherapy and significant variability within this clinical population, resulting in a stepwise trial and error approach. Consequently, EM is often associated with poorer health-related quality of life with higher morbidity. There is currently no consensus or guidelines on management of pain in EM. This is a review of the literature on management of pain using pharmacologic, procedural intervention and nonpharmacologic treatment in children and adults with EM.

Primary erythromelalgia – case report

“…This confirms the observations of Friborg et al that suggest that erythromelalgia is not an isolated disease but that it is a pattern of response of cutaneous microvasculature [4]. Tham and Giles rapported that erythromelalgia is associated with a neuropathy of small fibers and primary vasculopathies, characterized by an intermittent increase of blood flow, hypoxia, and possibly shunts, with increase in local cellular metabolism [5]. Due to the hypothesis of shunts, substances that alter the distribution of skin blood flow can improve the cutaneous oxygenation and induce symptoms relief [6].…”

Use of H-1 Antihistamine in Dermatology: More than Itch and Urticaria Control: A Systematic Review

“…The 5-HT and histamine antagonist cyproheptadine and pizotifen effectively relieved the burning pain and increased skin temperature [ 56 , 57 ]. A survey of the members of The Erythromelalgia Association reported marked improvement in 40% of patients with antihistamines, including desloratadine, chlorpheniramine, and diphenhydramine [ 58 ]. A child with erythromelalgia responded to cetirizine, and his symptoms aggravated once cetirizine was discontinued [ 59 ].…”

Painful and painless mutations of SCN9A and SCN11A voltage-gated sodium channels

“…Patients typically resort to foot lifting, cooling feet by fans or immersing them in water or iced water to reduce or relief pain. Although immersion in cold water is effective for mild cases, it can result in ulceration and maceration of foot skin leading to infection [19,116]. Recently, it has been reported that behavioural therapy reduced dependence on water immersion in PEM patients [67].…”

“…There is no consensus on pharmacotherapy. Among effective drugs are non-selective sodium blockers (lignocaine, mexiletine and carbamazepine) [ 82 , 116 ] which have been shown to inhibit Na V 1.7 [ 120 , 133 ].…”