{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T07:04:19Z","timestamp":1779951859226,"version":"3.53.1"},"reference-count":42,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T00:00:00Z","timestamp":1779926400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T00:00:00Z","timestamp":1779926400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Curr Pain Headache Rep"],"published-print":{"date-parts":[[2026,12]]},"DOI":"10.1007\/s11916-026-01514-3","type":"journal-article","created":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T06:30:39Z","timestamp":1779949839000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Erythromelalgia: Pathophysiology and Clinical Treatment Options, a Narrative Review"],"prefix":"10.1007","volume":"30","author":[{"given":"Riley K.","family":"Noble","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary O\u2019Dell","family":"Duplechin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed I.","family":"Anwar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles J.","family":"Fox","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sahar","family":"Shekoohi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan D.","family":"Kaye","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,5,28]]},"reference":[{"key":"1514_CR1","doi-asserted-by":"publisher","unstructured":"Eaton AC, Mayrovitz HN. Primary erythromelalgia: historical perspective and current update. Cureus. 2025;17(2):e78576. https:\/\/doi.org\/10.7759\/cureus.78576.","DOI":"10.7759\/cureus.78576"},{"key":"1514_CR2","doi-asserted-by":"publisher","unstructured":"Alhadad A, Wollmer P, Svensson A, Eriksson K-F. Erythromelalgia: incidence and clinical experience in a single centre in Sweden. Vasa. 2012;41(1):43\u20138. https:\/\/doi.org\/10.1024\/0301-1526\/a000162.","DOI":"10.1024\/0301-1526\/a000162"},{"key":"1514_CR3","doi-asserted-by":"publisher","unstructured":"Reed KB, Davis MDP. Incidence of erythromelalgia: a population-based study in Olmsted County, Minnesota. J Eur Acad Dermatol Venereol. 2009;23(1):13\u20135. https:\/\/doi.org\/10.1111\/j.1468-3083.2008.02938.x.","DOI":"10.1111\/j.1468-3083.2008.02938.x"},{"key":"1514_CR4","doi-asserted-by":"publisher","unstructured":"Caldito EG, Kaul S, Caldito NG, Piette W, Mehta S. Erythromelalgia. Part I: pathogenesis, clinical features, evaluation, and complications. J Am Acad Dermatol. 2024;90(3):453\u2013462. https:\/\/doi.org\/10.1016\/j.jaad.2023.02.071.","DOI":"10.1016\/j.jaad.2023.02.071"},{"key":"1514_CR5","doi-asserted-by":"publisher","unstructured":"Hoxha D, Bayro Jablonski NE, Chatterjee A, Abdallah J, Cartabuke R. Mimicking the usual suspects: erythromelalgia in the differential diagnosis, J Brown Hosp Med. 2025;4(3):137218. https:\/\/doi.org\/10.56305\/001c.137218.","DOI":"10.56305\/001c.137218"},{"key":"1514_CR6","unstructured":"Cascio MA, Mukhdomi T. Small fiber neuropathy. In StatPearls, Treasure Island (FL): StatPearls Publishing, 2026. [Online]. Available: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK582147\/.\u00a0\u00a0Accessed 6 May 2026."},{"key":"1514_CR7","doi-asserted-by":"publisher","unstructured":"Patel P, Zhang Y, Unikel LH, Edwards C. A case of sporadic erythromelalgia presenting with small fibre neuropathy. BMJ Case Rep. 2019;12(10):e230549. https:\/\/doi.org\/10.1136\/bcr-2019-230549.","DOI":"10.1136\/bcr-2019-230549"},{"key":"1514_CR8","doi-asserted-by":"publisher","unstructured":"Shastri A, Al Aiyan A, Kishore U, Farrugia ME. Immune-mediated neuropathies: pathophysiology and management. Int J Mol Sci. 2023;24(8):7288. https:\/\/doi.org\/10.3390\/ijms24087288.","DOI":"10.3390\/ijms24087288"},{"key":"1514_CR9","unstructured":"Edwards E, Yosipovitch G. Skin Manifestations of diabetes mellitus. In Endotext [Internet], MDText.com, Inc., 2025. [Online]. Available: https:\/\/www.ncbi.nlm.nih.gov\/sites\/books\/NBK481900\/.\u00a0Accessed 6 May 2026."},{"key":"1514_CR10","doi-asserted-by":"publisher","unstructured":"Zhang H, Qin Z, Feng X. Painful diabetic neuropathy complicated with secondary erythromelalgia in polycythemia vera: a case report. J Int Med Res. 2025;53(10):03000605251382382. https:\/\/doi.org\/10.1177\/03000605251382382.","DOI":"10.1177\/03000605251382382"},{"key":"1514_CR11","unstructured":"Davis D, Taqi M, Vasudevan A. Sciatica. In StatPearls, Treasure Island (FL): StatPearls Publishing, 2026. [Online]. Available: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK507908\/.\u00a0Accessed 6 May 2026."},{"key":"1514_CR12","unstructured":"Brown BD, Hood Watson KL. Cellulitis. In StatPearls, Treasure Island (FL): StatPearls Publishing, 2025. [Online]. Available: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK549770\/.\u00a0Accessed 12 Aug 2025."},{"key":"1514_CR13","doi-asserted-by":"publisher","unstructured":"Bruehl S. Complex regional pain syndrome. BMJ. 2015;351:h2730. https:\/\/doi.org\/10.1136\/bmj.h2730.","DOI":"10.1136\/bmj.h2730"},{"issue":"5","key":"1514_CR14","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1111\/j.1468-1331.2010.02947.x","volume":"17","author":"C Maih\u00f6fner","year":"2010","unstructured":"Maih\u00f6fner C, Seifert F, Markovic K. Complex regional pain syndromes: new pathophysiological concepts and therapies. Eur J Neurol. 2010;17(5):649\u201360. https:\/\/doi.org\/10.1111\/j.1468-1331.2010.02947.x.","journal-title":"Eur J Neurol"},{"key":"1514_CR15","doi-asserted-by":"publisher","unstructured":"Suresh E. Diagnostic approach to patients with suspected vasculitis. Postgrad Med J. 2006;82(970):483\u2013488. https:\/\/doi.org\/10.1136\/pgmj.2005.042648.","DOI":"10.1136\/pgmj.2005.042648"},{"key":"1514_CR16","doi-asserted-by":"crossref","unstructured":"Dicks K, Rizek P. Massage therapy techniques as pain management for erythromelalgia: a case report. Int J Ther Massage Bodywork. 2010;3(4):5\u20139.","DOI":"10.3822\/ijtmb.v3i4.83"},{"key":"1514_CR17","doi-asserted-by":"publisher","unstructured":"Lee JU et al. Dec., Procedural interventions for erythromelalgia: a narrative review. Vasc Med. 2024;29(6):723\u2013732. https:\/\/doi.org\/10.1177\/1358863X241279427.","DOI":"10.1177\/1358863X241279427"},{"key":"1514_CR18","doi-asserted-by":"publisher","first-page":"1689","DOI":"10.2147\/JPR.S154462","volume":"11","author":"SW Tham","year":"2018","unstructured":"Tham SW, Giles M. Current pain management strategies for patients with erythromelalgia: a critical review. J Pain Res. 2018;11:1689\u201398. https:\/\/doi.org\/10.2147\/JPR.S154462.","journal-title":"J Pain Res"},{"issue":"1","key":"1514_CR19","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1590\/abd1806-4841.20187535","volume":"93","author":"MB Leroux","year":"2018","unstructured":"Leroux MB. Erythromelalgia: a cutaneous manifestation of neuropathy? Bras Dermatol. 2018;93(1):86\u201394. https:\/\/doi.org\/10.1590\/abd1806-4841.20187535.","journal-title":"Bras Dermatol"},{"issue":"5","key":"1514_CR20","first-page":"E658","volume":"17","author":"AFG da Costa","year":"2014","unstructured":"da Costa AFG, Meireles J, Festas MJ, Gomes A, Abreu P. Therapeutic success with local botulinum toxin in erythromelalgia. Pain Physician. 2014;17(5):E658\u2013660.","journal-title":"Pain Physician"},{"key":"1514_CR21","doi-asserted-by":"crossref","unstructured":"D\u2019Angelo R, Cohen IT, Brandom BW. Continuous epidural infusion of bupivacaine and fentanyl for erythromelalgia in an adolescent. Anesth Analg. 1992;74(1):142\u2013144.","DOI":"10.1213\/00000539-199201000-00024"},{"key":"1514_CR22","doi-asserted-by":"publisher","unstructured":"Hagedorn JM, Canzanello N, Lamer TJ. Dorsal root ganglion stimulation for erythromelalgia related foot pain: a case report and review of the literature. Pain Pract. 2021;21(6):698\u2013702. https:\/\/doi.org\/10.1111\/papr.12998.","DOI":"10.1111\/papr.12998"},{"key":"1514_CR23","unstructured":"Jha SK, Karna B, Goodman MB. Erythromelalgia. In StatPearls, Treasure Island (FL): StatPearls Publishing, 2025. [Online]. Available: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK557787\/.\u00a0Accessed 16 Aug 2025."},{"key":"1514_CR24","doi-asserted-by":"publisher","unstructured":"Tang Z, Chen Z, Tang B, Jiang H. Primary erythromelalgia: a review. Orphanet J Rare Dis. 2015;10:127. https:\/\/doi.org\/10.1186\/s13023-015-0347-1.\u00a0","DOI":"10.1186\/s13023-015-0347-1"},{"key":"1514_CR25","doi-asserted-by":"publisher","unstructured":"Magro C, Kalomeris T, Guo L. The cutaneous pathology of erythromelalgia and its role in establishing critical clues regarding pathogenesis. Am J Dermatopathol. 2025;47(3):184\u2013190. https:\/\/doi.org\/10.1097\/DAD.0000000000002912.","DOI":"10.1097\/DAD.0000000000002912"},{"key":"1514_CR26","doi-asserted-by":"publisher","unstructured":"Sheets PL, Jackson JO, Waxman SG, Dib-Hajj SD, Cummins TR. A Nav1.7 channel mutation associated with hereditary erythromelalgia contributes to neuronal hyperexcitability and displays reduced lidocaine sensitivity. J Physiol. 2007;581(Pt 3):1019\u20131031. https:\/\/doi.org\/10.1113\/jphysiol.2006.127027.","DOI":"10.1113\/jphysiol.2006.127027"},{"key":"1514_CR27","doi-asserted-by":"publisher","unstructured":"Yang Y, et al. Nav1.7-A1632G mutation from a family with inherited erythromelalgia: enhanced firing of dorsal root ganglia neurons evoked by thermal stimuli. J Neurosci. 2016;36(28):7511\u201322. https:\/\/doi.org\/10.1523\/JNEUROSCI.0462-16.2016.","DOI":"10.1523\/JNEUROSCI.0462-16.2016"},{"key":"1514_CR28","doi-asserted-by":"publisher","unstructured":"Algarni AS, et al. Comparative efficacy and tolerability of treatments for erythromelalgia: a systematic review. Med (Kaunas). 2025;61(5):920. https:\/\/doi.org\/10.3390\/medicina61050920.","DOI":"10.3390\/medicina61050920"},{"key":"1514_CR29","doi-asserted-by":"publisher","unstructured":"Kalgaard OM, Clausen OP, Mellbye OJ, Hovig T, Kvernebo K. Nonspecific capillary proliferation and vasculopathy indicate skin hypoxia in erythromelalgia. Arch Dermatol. 2011;147(3):309\u2013314. https:\/\/doi.org\/10.1001\/archdermatol.2010.337.","DOI":"10.1001\/archdermatol.2010.337"},{"key":"1514_CR30","doi-asserted-by":"publisher","unstructured":"Eaton AC, Mayrovitz HN. Primary erythromelalgia: historical perspective and current update. Cureus. 2025;17(2):e78576. https:\/\/doi.org\/10.7759\/cureus.78576.","DOI":"10.7759\/cureus.78576"},{"key":"1514_CR31","doi-asserted-by":"publisher","unstructured":"Kang BC, Nam DJ, Ahn EK, Yoon DM, Cho JG. Secondary erythromelalgia - a case report -. Korean J Pain. 2013;26(3):299\u2013302. https:\/\/doi.org\/10.3344\/kjp.2013.26.3.299.","DOI":"10.3344\/kjp.2013.26.3.299"},{"key":"1514_CR32","doi-asserted-by":"crossref","unstructured":"Kurzrock R, Cohen PR. Erythromelalgia and myeloproliferative disorders. Arch Intern Med. 1989;149(1):105\u2013109.","DOI":"10.1001\/archinte.1989.00390010109013"},{"key":"1514_CR33","doi-asserted-by":"publisher","unstructured":"Caldito EG, Caldito NG, Kaul S, Piette W, Mehta S. Erythromelalgia. Part II: differential diagnoses and management. J Am Acad Dermatol. 2024;90(3):465\u2013474. https:\/\/doi.org\/10.1016\/j.jaad.2023.02.070.","DOI":"10.1016\/j.jaad.2023.02.070"},{"key":"1514_CR34","doi-asserted-by":"publisher","unstructured":"Herrick AL, Wigley FM. Raynaud\u2019s phenomenon. Best Pract Res Clin Rheumatol. 2020;34(1):101474. https:\/\/doi.org\/10.1016\/j.berh.2019.101474.\u00a0","DOI":"10.1016\/j.berh.2019.101474"},{"issue":"3","key":"1514_CR35","doi-asserted-by":"publisher","first-page":"e20240035","DOI":"10.1590\/2175-8239-JBN-2024-0035en","volume":"46","author":"JT do Monte Neto","year":"2024","unstructured":"do Monte Neto JT, Kirsztajn GM. The role of podocyte injury in the pathogenesis of Fabry disease nephropathy. J Bras Nefrol. 2024;46(3):e20240035. https:\/\/doi.org\/10.1590\/2175-8239-JBN-2024-0035en.","journal-title":"J Bras Nefrol"},{"key":"1514_CR36","doi-asserted-by":"publisher","unstructured":"Ge W, et al. A novel mutation of \u03b1-galactosidase A gene causes Fabry disease mimicking primary erythromelalgia in a Chinese family. Int J Neurosci. 2017;127(5):448\u201353. https:\/\/doi.org\/10.1080\/00207454.2016.1191483.","DOI":"10.1080\/00207454.2016.1191483"},{"key":"1514_CR37","doi-asserted-by":"publisher","unstructured":"Fooladi S, Hasoon J, Kaye AD, Abd-Elsayed A. Diagnostic criteria and technical evaluation of complex regional pain syndrome: a narrative review. Diagnostics (Basel). 2025;15(17):2281. https:\/\/doi.org\/10.3390\/diagnostics15172281.","DOI":"10.3390\/diagnostics15172281"},{"key":"1514_CR38","doi-asserted-by":"publisher","unstructured":"Thami GP, Bhalla M. Erythromelalgia induced by possible calcium channel blockade by ciclosporin. BMJ. 2003;326(7395):910. https:\/\/doi.org\/10.1136\/bmj.326.7395.910.","DOI":"10.1136\/bmj.326.7395.910"},{"key":"1514_CR39","doi-asserted-by":"publisher","unstructured":"Herrick AL, Murray A. The role of capillaroscopy and thermography in the assessment and management of Raynaud\u2019s phenomenon. Autoimmun Rev. 2018;17(5):465\u201372. https:\/\/doi.org\/10.1016\/j.autrev.2017.11.036.","DOI":"10.1016\/j.autrev.2017.11.036"},{"key":"1514_CR40","doi-asserted-by":"publisher","unstructured":"Tolley JA, Walsh LE. Primary erythromelalgia treated with 10% capsaicin cream: a case report and a 10-year follow-up. Cureus. 2022;14(8):e28342. https:\/\/doi.org\/10.7759\/cureus.28342.","DOI":"10.7759\/cureus.28342"},{"key":"1514_CR41","doi-asserted-by":"publisher","unstructured":"Ma JE, Lee JUJ, Sartori-Valinotti JC, Rooke TW, Sandroni P, Davis MDP. Erythromelalgia: a review of medical management options and our approach to management. Mayo Clin Proc. 2023;98(1):136\u2013149. https:\/\/doi.org\/10.1016\/j.mayocp.2022.08.005.","DOI":"10.1016\/j.mayocp.2022.08.005"},{"key":"1514_CR42","doi-asserted-by":"publisher","unstructured":"Chakravarty K, Pharoah PD, Scott DG, Barker S. Erythromelalgia\u2013the role of hypnotherapy. Postgrad Med J. 1992;68(795):44\u20136. https:\/\/doi.org\/10.1136\/pgmj.68.795.44.","DOI":"10.1136\/pgmj.68.795.44"}],"container-title":["Current Pain and Headache Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11916-026-01514-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11916-026-01514-3","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11916-026-01514-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T06:30:43Z","timestamp":1779949843000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s11916-026-01514-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,28]]},"references-count":42,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,12]]}},"alternative-id":["1514"],"URL":"https:\/\/doi.org\/10.1007\/s11916-026-01514-3","relation":{},"ISSN":["1531-3433","1534-3081"],"issn-type":[{"value":"1531-3433","type":"print"},{"value":"1534-3081","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,28]]},"assertion":[{"value":"29 September 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 May 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 May 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This article is based on previous studies and contains no new studies with human participants or animals performed by any authors.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Human Participants"}},{"value":"ADK is the editor in Chief for CPHRs.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"77"}}