Erythromelalgia. Part I: Pathogenesis, clinical features, evaluation, and complications

J Am Acad Dermatol. 2024 Mar;90(3):453-462. doi: 10.1016/j.jaad.2023.02.071. Epub 2023 Jun 24.

Abstract

Erythromelalgia is a rare pain disorder that is underrecognized and difficult-to-treat. It is characterized by episodes of extremity erythema and pain that can be disabling; it may be genetic, related to an underlying systemic disease, or idiopathic. Considering the prominent cutaneous features characteristic of the condition, dermatologists can play an important role in early recognition and limitation of morbidity. The first article in this 2-part continuing medical education series reviews the epidemiology, pathogenesis, clinical manifestations, evaluation, and complications.

Keywords: Raynaud’s phenomenon; SCN9A gene; complex regional pain syndrome; erythermalgia; erythromelalgia; red ear syndrome; voltage gated sodium channel.

Publication types

  • Review

MeSH terms

  • Erythema
  • Erythromelalgia* / diagnosis
  • Erythromelalgia* / epidemiology
  • Erythromelalgia* / etiology
  • Humans
  • Pain / diagnosis
  • Pain / etiology
  • Skin / pathology