title: Pediatric erythromelalgia: a retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period.
abstract: BACKGROUND
  Erythromelalgia has not been well characterized in the pediatric population.
  
  
  OBJECTIVE
  We sought to review our experience of erythromelalgia in the pediatric age group.
  
  
  METHODS
  We conducted a retrospective review of patients 18 years of age and younger with a diagnosis of erythromelalgia who were examined at Mayo Clinic in Rochester, MN, from 1970 to 2007.
  
  
  RESULTS
  The records of 32 patients (girls, 22 [69%]) were evaluated. Mean age was 14.1 years (range, 5-18 years) and mean time to diagnosis was 5.2 years. Seven patients (22%) had a first-degree relative with erythromelalgia; 4 were from the same family. Physical activity was limited because of discomfort in 21 patients (66%) and school attendance was affected in 11 patients (34%). Noninvasive vascular studies, which compared temperature, laser Doppler flow, and transcutaneous oximetry in the toes, identified vascular abnormalities in 13 (93%) of 14 patients. Neurophysiologic studies with autonomic reflex screening (including quantitative sudomotor axon reflex test and thermoregulatory sweat testing) showed evidence of a small-fiber neuropathy involving the skin in 10 (59%) of 17 patients studied; there was no evidence of large-fiber neuropathy in 20 patients in whom electromyographic and nerve conduction studies were performed. Topical lidocaine was the most commonly prescribed treatment (44%). Fifteen patients were monitored for an average of 9.1 years (median, 5.0 years; range, 0.4-23.7 years). At last follow-up, 5 patients had stable disease, 4 showed improvement, two had resolution, one reported worsening of symptoms, and 3 had died (one suicide).
  
  
  CONCLUSIONS
  Conclusions are limited because this was a retrospective chart review.
  
  
  CONCLUSIONS
  Erythromelalgia in pediatric patients is associated with substantial morbidity and even death. The majority of cases are not inherited. Most patients studied have associated small-fiber neuropathy. The disease course is variable. A reliable and safe treatment has not been determined.
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
authors.forename: Robert H
authors.initials: RH
authors.lastname: Cook-Norris
authors.forename: Megha M
authors.initials: MM
authors.lastname: Tollefson
authors.forename: Andres E
authors.initials: AE
authors.lastname: Cruz-Inigo
authors.forename: Paola
authors.initials: P
authors.lastname: Sandroni
authors.forename: Mark D P
authors.initials: MD
authors.lastname: Davis
authors.forename: Dawn M R
authors.initials: DM
authors.lastname: Davis
mesh_terms: D000293:Adolescent; D000779:Anesthetics, Local / Q000627:therapeutic use; D002481:Cellulitis / Q000175:diagnosis / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D018450:Disease Progression; D004576:Electromyography; D004916:Erythromelalgia / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008012:Lidocaine / Q000627:therapeutic use*; D008297:Male; D009412:Nerve Fibers / Q000502:physiology; D009431:Neural Conduction; D010092:Oximetry; D011928:Raynaud Disease / Q000175:diagnosis / Q000401:mortality; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000779:Anesthetics, Local; D008012:Lidocaine