Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review

Don Daniel Ocay^{1

2}, Maria Graziano Maloney^{3

4}, Genevieve D'Souza⁵, Catherine A Brownstein^{6

7}, Jacqui Clinch⁸, Dawn Marie Davis^{9

10}, Deirdre De Ranieri¹¹, Carolina Donado^{3

12}, Meghan Halpin³, Deepa Kattail¹³, Benjamin Howard Lee¹⁴, Kimberly Lobo³, Danielle Ravetti^{15

16}, Paola Sandroni¹⁷, Jennifer N Stinson^{13

18}, See Wan Tham¹⁹, Gary A Walco^{19

20}, Suellen M Walker^{21

22}, Timothy W Yu^{6

7}, Charles B Berde^{3

12}

Affiliations

¹ Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, USA. dondaniel.ocay@childrens.harvard.edu.
² Department of Anaesthesia, Harvard Medical School, Boston, MA, USA. dondaniel.ocay@childrens.harvard.edu.
³ Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, USA.
⁴ F.M. Kirby Neurobiology Center, Boston Children's Hospital, Boston, MA, USA.
⁵ Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University, Stanford, CA, USA.
⁶ Division of Genetics and Genomics, Department of Pediatrics, Boston Children's Hospital, Boston, MA, USA.
⁷ Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
⁸ Department of Pediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, United Kingdom.
⁹ Department of Dermatology, Mayo Clinic Rochester, Rochester, MN, USA.
¹⁰ Department of Pediatric and Adolescent Medicine, Mayo Clinic Rochester, Rochester, MN, USA.
¹¹ Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
¹² Department of Anaesthesia, Harvard Medical School, Boston, MA, USA.
¹³ Department of Anesthesia and Pain Medicine, The Hospital for Sick Children (Sickkids), Toronto, ON, Canada.
¹⁴ Department of Anesthesiology, Emory University School of Medicine, Atlanta, GA, USA.
¹⁵ Patient Partner, Lodi, CA, USA.
¹⁶ The Erythromelalgia Association, Lodi, CA, USA.
¹⁷ Department of Neurology, Mayo Clinic Rochester, Rochester, MN, USA.
¹⁸ Research Institute, The Hospital for Sick Children (SickKids), Toronto, ON, Canada.
¹⁹ Department of Anesthesiology and Pain Medicine, University of Washington School of Medicine, Seattle, WA, USA.
²⁰ Department of Anesthesiology and Pain Medicine, Seattle Children's Hospital, Seattle, WA, USA.
²¹ Developmental Neurosciences, University College London Great Ormond Street Institute of Child Health, London, United Kingdom.
²² Department of Paediatric Anaesthesia and Pain Medicine, Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.

PMID: 39821136
PMCID: PMC12507648
DOI: 10.1038/s41390-025-03817-4

Review

Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review

Don Daniel Ocay et al. Pediatr Res. 2025 Sep.

. 2025 Sep;98(3):786-799.

doi: 10.1038/s41390-025-03817-4. Epub 2025 Jan 16.

Authors

Affiliations

¹ Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, USA. dondaniel.ocay@childrens.harvard.edu.
² Department of Anaesthesia, Harvard Medical School, Boston, MA, USA. dondaniel.ocay@childrens.harvard.edu.
³ Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, USA.
⁴ F.M. Kirby Neurobiology Center, Boston Children's Hospital, Boston, MA, USA.
⁵ Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University, Stanford, CA, USA.
⁶ Division of Genetics and Genomics, Department of Pediatrics, Boston Children's Hospital, Boston, MA, USA.
⁷ Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
⁸ Department of Pediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, United Kingdom.
⁹ Department of Dermatology, Mayo Clinic Rochester, Rochester, MN, USA.
¹⁰ Department of Pediatric and Adolescent Medicine, Mayo Clinic Rochester, Rochester, MN, USA.
¹¹ Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
¹² Department of Anaesthesia, Harvard Medical School, Boston, MA, USA.
¹³ Department of Anesthesia and Pain Medicine, The Hospital for Sick Children (Sickkids), Toronto, ON, Canada.
¹⁴ Department of Anesthesiology, Emory University School of Medicine, Atlanta, GA, USA.
¹⁵ Patient Partner, Lodi, CA, USA.
¹⁶ The Erythromelalgia Association, Lodi, CA, USA.
¹⁷ Department of Neurology, Mayo Clinic Rochester, Rochester, MN, USA.
¹⁸ Research Institute, The Hospital for Sick Children (SickKids), Toronto, ON, Canada.
¹⁹ Department of Anesthesiology and Pain Medicine, University of Washington School of Medicine, Seattle, WA, USA.
²⁰ Department of Anesthesiology and Pain Medicine, Seattle Children's Hospital, Seattle, WA, USA.
²¹ Developmental Neurosciences, University College London Great Ormond Street Institute of Child Health, London, United Kingdom.
²² Department of Paediatric Anaesthesia and Pain Medicine, Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.

PMID: 39821136
PMCID: PMC12507648
DOI: 10.1038/s41390-025-03817-4

Abstract

Erythromelalgia is a rare, chronic pain disorder characterized by the triad of intense burning sensation, warmth, and redness, primarily involving the hands and feet, and usually alleviated by cold and worsened by heat. The objective of this scoping review was to: 1) map the existing literature on erythromelalgia in youth, 2) identify knowledge gaps, and 3) inform directions for future research in pediatric erythromelalgia. One hundred and sixty-seven studies reporting 411 cases of childhood-onset erythromelalgia were identified. Variability was found in reporting of clinical symptoms, the clinical presentations and diagnostic criteria used for classification of erythromelagia, the clinical assessments and investigations performed, and the types of interventions and management plans utilised. While factors to aid early recognition and optimize management have been identified, there are also significant gaps for future research to address. Ongoing efforts to develop a multicenter registry of pediatric erythromelalgia cases, with standardized data collection and reporting, will be beneficial to establish consensus recommendations for the diagnosis and management of pediatric erythromelalgia. IMPACT: This scoping review maps the existing literature on pediatric erythromelalgia. Variability was found in reporting of clinical symptoms, the clinical presentations and diagnostic criteria used for classification of erythromelagia, the clinical assessments and investigations performed, and the types of interventions and management plans utilised. The development of an international registry would immensely benefit multidisciplinary experts involved in the care of pediatric erythromelalgia and those with lived experience.

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Conflict of interest statement

Competing interests: The authors declare no competing interests.

Figures

**Fig. 1. Proposal for diagnostic workup and classification for pediatric erythromelalgia and treatment approach.**
Burning pain, redness, and increased temperature of the affected skin can be either continuous or episodic.

See this image and copyright information in PMC

References

1. Mitchell, S. W. On a Rare Vaso-Motor Neurosis of the Extremities, and on the Maladies with Which It May Be Confounded ([publisher not identified], 1878).
1. Brown, G. E. Erythromelalgia and other disturbances of the extremities accompanied by vasodilatation and burning. Am. J. Med. Sci.183, 12 (1932).
1. Smith, L. A. & Allen, E. V. Erythermalgia (erythromelalgia) of the extremities - a syndrome characterized by redness, heat, and pain. Am. Heart J.16, 175–188 (1938).
1. Drenth, J. P. & Michiels, J. J. Erythromelalgia and erythermalgia: diagnostic differentiation. Int. J. Dermatol33, 393–397 (1994). - PubMed
1. Arthur, L. et al. Pediatric erythromelalgia and Scn9a mutations: systematic review and single-center case series. J. Pediatr.206, 217–224 e219 (2019). - PubMed

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Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review

Affiliations

Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review

Authors

Affiliations

Abstract

Conflict of interest statement

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References

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources