Erythromelalgia in an adolescent
DOI:
https://doi.org/10.35954/SM2026.45.1.9.e503Keywords:
Early diagnosis, pain, erythema, erythromelalgia, follow-up studies, gabapentin, treatment outcomeAbstract
Introduction: Erythromelalgia is a rare condition that is difficult to diagnose and treat. It primarily affects the hands and feet and is characterized by intense, burning pain, heat, and flushing that occurs in episodes and significantly impairs quality of life.
Objective: To highlight the importance of recognizing the clinical manifestations of this rare disease in order to initiate early therapeutic measures with the aim of increasing the survival probability of affected patients.
Clinical case: adolescent with symptoms in the hands who was diagnosed with erythromelalgia six years after the onset of episodes. We describe the follow-up of the case over a period of just over one year, the treatments that were attempted without success, and the treatment that has so far provided symptom relief.
Discussion: We emphasize the difficulty in diagnosing and treating this condition, with the aim of collaborating with other professionals to facilitate early diagnosis and timely treatment.
NOTE: This article has been approved by the Editorial Committee.
Received for review: September 2025.
Accepted for publication: December 2025.
Publication date: March 2026.
Correspondence: Central Hospital of the Armed Forces. Av. Centenario 3057. Zip Code 11600. Tel.: (+598) 2487 66 66. Montevideo, Uruguay.
Contact email: sofiabergeret@gmail.com
Downloads
Metrics
References
Davis MD, O'Fallon WM, Rogers RS 3rd, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol. 2000 Mar; 136(3):330-6. https://doi.org/10.1001/archderm.136.3.330 PMID: 10724194. DOI: https://doi.org/10.1001/archderm.136.3.330
Leroux MB. Erythromelalgia: a cutaneous manifestation of neuropathy? An Bras Dermatol. 2018 Jan-Feb; 93(1):86-94. https://doi.org/10.1590/abd1806-4841.20187535 PMID: 29641704; PMCID: PMC5871369. DOI: https://doi.org/10.1590/abd1806-4841.20187535
Tham SW, Li L, Effraim P, Waxman S. Between fire and ice: refractory hypothermia and warmth-induced pain in inherited erythromelalgia. BMJ Case Rep. 2017 Jul 26; 2017:bcr2017219486. https://doi.org/10.1136/bcr-2017-219486 PMID: 28751508; PMCID: PMC5612348. DOI: https://doi.org/10.1136/bcr-2017-219486
Sousa RÁP, Vasconcelos LRS, Sá MVBO. Intravenous immunoglobulin therapy in erythromelalgia management: a case report. Einstein (Sao Paulo). 2025 Mar 3; 23:eRC1236. https://doi.org/10.31744/einstein_journal/2025RC1236 PMID: 40053049; PMCID: PMC11869793. DOI: https://doi.org/10.31744/einstein_journal/2025RC1236
Parker LK, Ponte C, Howell KJ, Ong VH, Denton CP, Schreiber BE. Clinical features and management of erythromelalgia: long term follow-up of 46 cases. Clin Exp Rheumatol. 2017 Jan-Feb; 35(1):80-84. Epub 2016 Aug 2. PMID: 27494156.
Tham SW, Giles M. Current pain management strategies for patients with erythromelalgia: a critical review. J Pain Res. 2018 Aug 30; 11:1689-1698. https://doi.org/10.2147/JPR.S154462 PMID: 30214279; PMCID: PMC6121769. DOI: https://doi.org/10.2147/JPR.S154462
Tang Z, Chen Z, Tang B, Jiang H. Primary erythromelalgia: a review. Orphanet J Rare Dis. 2015 Sep 30; 10:127. https://doi.org/10.1186/s13023-015-0347-1 PMID: 26419464; PMCID: PMC4589109. DOI: https://doi.org/10.1186/s13023-015-0347-1
Gil F, Luís P, Duarte M, Martins C. Eritromelalgia secundaria: informe de caso. Actas Dermosifiliogr. 2020; 111(10):885-886. https://doi.org/10.1016/j.ad.2019.01.027 DOI: https://doi.org/10.1016/j.ad.2019.01.027
Reed KB, Davis MD. Incidence of erythromelalgia: a population-based study in Olmsted County, Minnesota. J Eur Acad Dermatol Venereol. 2009 Jan; 23(1):13-5. https://doi.org/10.1111/j.1468-3083.2008.02938.x Epub 2008 Aug 18. PMID: 18713229; PMCID: PMC2771547. DOI: https://doi.org/10.1111/j.1468-3083.2008.02938.x
Febles Sanabria RJ, Garcia Herrera AL, Rodriguez Pereira M, Carreño Rolando IE, Pérez Martín O. Eritromelalgia. Informe de un caso. Rev Méd Electrón [Internet]. 10 de noviembre de 2020 [citado 10 de octubre de 2025]; 42(6):1-8. Disponible en: https://revmedicaelectronica.sld.cu/index.php/rme/article/view/3295
Davis MD, Sandroni P, Rooke TW, Low PA. Erythromelalgia: vasculopathy, neuropathy, or both? A prospective study of vascular and neurophysiologic studies in erythromelalgia. Arch Dermatol. 2003 Oct; 139(10):1337-43. https://doi.org/10.1001/archderm.139.10.1337 PMID: 14568838. DOI: https://doi.org/10.1001/archderm.139.10.1337
Loureiro MP, Novais PM, Paulin JAN, de Almeida DB, de Lemos AN. Endoscopic lumbar sympathectomy as a treatment option for primary erythromelalgia - case report and review. J Vasc Bras. 2023 Mar 10; 22:e20220095. https://doi.org/10.1590/1677-5449.202200952 PMID: 36950140; PMCID: PMC10026352. DOI: https://doi.org/10.1590/1677-5449.202200952
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 Sofía Bergeret, María Fernanda Cruz Pereira , Patsy González, Silvina Lorena Olivera Silva
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Until 2024 we use the Creative Commons Attribution/NonCommercial Attribution 4.0 International License https://creativecommons.org/licenses/by-nc/4.0/deed.es. Which states that: you are free to share, copy and redistribute the material in any medium or format, as well as to adapt, remix, transform and build upon the material. Under the following terms:
Attribution: you must give proper credit , provide a link to the license, and indicate if changes have been made . You may do so in any reasonable manner, but not in such a way as to suggest that you or your use is endorsed by the licensor.
NonCommercial: you may not use the material for commercial purposes.
As of 2025 authors retain their copyright and assign to the journal the right of first publication of their work, which shall simultaneously be subject to the license https://creativecommons.org/licenses/by-nc-sa/4.0/deed.es that permits sharing, copying and redistribution of the material in any medium or format provided that initial publication in this journal is indicated. Adapt, remix, transform and build upon the material. If you remix, transform, or build from the material, you must distribute your contribution under the same license as the original and may not make use of the material for commercial purposes.
Under the following terms:
1. Attribution: you must give proper credit, provide a link to the license, and indicate whether changes have been made. You may do so in any reasonable manner, but not in such a way as to suggest that you or your use is endorsed by the licensor.
2. NonCommercial: you may not use the material for commercial purposes.
3. ShareAlike: if you remix, transform or build upon the material, you must distribute your contribution under the same license as the original.
PlumX Metrics
/
