Defining a treatable cause of erythromelalgia: acute adolescent autoimmune small-fiber axonopathy

Anesth Analg. 2007 Feb;104(2):438-41. doi: 10.1213/01.ane.0000252965.83347.25.


Conditions described as "erythromelalgia" and "erythermalgia" are being formally specified by etiological diagnoses that enable the use of disease-modifying as well as symptomatic treatments. We describe an otherwise healthy 20-year-old man with acute-onset erythromelalgia. Severe bilateral distal limb pain and vasodilation persisted despite the use of many antihyperalgesics. Pathological examination of cutaneous nerve endings revealed severe small-fiber predominant axonopathy. Treatment of his apparent autoimmune polyneuropathy with high dose corticosteroids, 4 days of lidocaine infusion, and a prednisone taper cured him. Similarities to other cases allowed us to tentatively characterize a new treatable cause of erythromelalgia; acute adolescent autoimmune small-fiber axonopathy. In this report we evaluate various options for diagnosis and treatment.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Analgesics / therapeutic use
  • Axons / pathology*
  • Erythromelalgia / etiology*
  • Erythromelalgia / pathology
  • Erythromelalgia / therapy*
  • Guillain-Barre Syndrome / complications*
  • Guillain-Barre Syndrome / pathology
  • Guillain-Barre Syndrome / therapy*
  • Humans
  • Male
  • Nerve Endings / pathology*


  • Adrenal Cortex Hormones
  • Analgesics