Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier

Introduction: Linear scleroderma en coup de sabre is a subtype of scleroderma hallmarked by cutaneous and extracutaneous manifestations in which neurological symptoms can be a predominant feature of this condition. Case presentation: We report a case of a previously healthy 47-year old male who deve...

Full description

Autores:: Sanguino-Caneva, Ciro
Carrillo-Chapman, Carlos
Luque-Llano, Melissa
Bermúdez, Valmore
Vargas-Manotas, José

Tipo de recurso:

Fecha de publicación:: 2023

Institución:: Universidad Simón Bolívar

Repositorio:: Repositorio Digital USB

Idioma:: eng

id	USIMONBOL2_b345ef88bcd158354d24aad5ea81787b
oai_identifier_str	oai:bonga.unisimon.edu.co:20.500.12442/16804
network_acronym_str	USIMONBOL2
network_name_str	Repositorio Digital USB
repository_id_str
dc.title.eng.fl_str_mv	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
title	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
spellingShingle	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier Localized Scleroderma Linear Scleroderma en coup de sabre Morphea Neurologic involvement Neurologic manifestations Prefrontal syndrome
title_short	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
title_full	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
title_fullStr	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
title_full_unstemmed	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
title_sort	Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
dc.creator.fl_str_mv	Sanguino-Caneva, Ciro Carrillo-Chapman, Carlos Luque-Llano, Melissa Bermúdez, Valmore Vargas-Manotas, José
dc.contributor.author.none.fl_str_mv	Sanguino-Caneva, Ciro Carrillo-Chapman, Carlos Luque-Llano, Melissa Bermúdez, Valmore Vargas-Manotas, José
dc.subject.keywords.eng.fl_str_mv	Localized Scleroderma Linear Scleroderma en coup de sabre Morphea Neurologic involvement Neurologic manifestations Prefrontal syndrome
topic	Localized Scleroderma Linear Scleroderma en coup de sabre Morphea Neurologic involvement Neurologic manifestations Prefrontal syndrome
description	Introduction: Linear scleroderma en coup de sabre is a subtype of scleroderma hallmarked by cutaneous and extracutaneous manifestations in which neurological symptoms can be a predominant feature of this condition. Case presentation: We report a case of a previously healthy 47-year old male who developed neuropsychiatric symptoms and right-sided cephalalgia for two months. Clinical examination revealed a right frontoparietal cutaneous lesion and neurological findings suggesting a medial prefrontal syndrome. The neuroimaging evaluation identified scalp and bone thinning adjacent to the skin lesion and cortical subcortical white matter hyperintensity due to vasogenic oedema at the right frontal and parietal region. A biopsy from the affected area revealed reactive gliosis. Conclusion: To our knowledge, this is the first linear scleroderma en coup de sabre report associated with a neurological involvement typical of a medial prefrontal syndrome. This case highlights the importance of clinical acuity in recognising atypical phenotypes within the spectrum of this uncommon disease.
publishDate	2023
dc.date.issued.none.fl_str_mv	2023
dc.date.accessioned.none.fl_str_mv	2025-07-07T22:01:55Z
dc.date.available.none.fl_str_mv	2025-07-07T22:01:55Z
dc.type.coarversion.fl_str_mv	http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.coar.fl_str_mv	http://purl.org/coar/resource_type/c_2df8fbb1
dc.type.driver.none.fl_str_mv	info:eu-repo/semantics/article
dc.type.spa.none.fl_str_mv	Artículo científico
dc.identifier.citation.spa.fl_str_mv	Sanguino-Caneva C, Carrillo-Chapman C, Luque-Llano M et al. Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier [version 1; peer review: 1 approved with reservations, 1 not approved] F1000Research 2023, 12:1254 https://doi.org/10.12688/f1000research.141188.1
dc.identifier.issn.none.fl_str_mv	20461402
dc.identifier.uri.none.fl_str_mv	https://hdl.handle.net/20.500.12442/16804
dc.identifier.doi.none.fl_str_mv	https://doi.org/10.12688/f1000research.141188.1
dc.identifier.url.none.fl_str_mv	https://f1000research.com/articles/12-1254/v1
identifier_str_mv	Sanguino-Caneva C, Carrillo-Chapman C, Luque-Llano M et al. Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier [version 1; peer review: 1 approved with reservations, 1 not approved] F1000Research 2023, 12:1254 https://doi.org/10.12688/f1000research.141188.1 20461402
url	https://hdl.handle.net/20.500.12442/16804 https://doi.org/10.12688/f1000research.141188.1 https://f1000research.com/articles/12-1254/v1
dc.language.iso.eng.fl_str_mv	eng
language	eng
dc.rights.eng.fl_str_mv	Attribution-NonCommercial-NoDerivatives 4.0 International
dc.rights.coar.fl_str_mv	http://purl.org/coar/access_right/c_abf2
dc.rights.uri.none.fl_str_mv	http://creativecommons.org/licenses/by-nc-nd/4.0/
dc.rights.accessrights.none.fl_str_mv	info:eu-repo/semantics/openAccess
rights_invalid_str_mv	Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/ http://purl.org/coar/access_right/c_abf2
eu_rights_str_mv	openAccess
dc.format.mimetype.none.fl_str_mv	pdf
dc.publisher.eng.fl_str_mv	Taylor and Francis
dc.publisher.spa.fl_str_mv	Facultad de Ciencias de la Salud
dc.source.spa.fl_str_mv	F1000Research Vol. 12 No. 1254, (2023)
institution	Universidad Simón Bolívar
bitstream.url.fl_str_mv	https://bonga.unisimon.edu.co/bitstreams/2aaba2f9-d690-4df2-8f8c-fa26be22c4fd/download https://bonga.unisimon.edu.co/bitstreams/d3db64e3-9315-44fb-962a-5eb494ccba2e/download https://bonga.unisimon.edu.co/bitstreams/7ac18877-adae-4c9c-a0df-8851e5d0e306/download https://bonga.unisimon.edu.co/bitstreams/476b2dd9-0e48-4ef9-953f-96d43708615f/download https://bonga.unisimon.edu.co/bitstreams/42f9e337-d77d-43a9-a366-595b66f8f00e/download
bitstream.checksum.fl_str_mv	626a1b87b62bc76d308ee3213e6c6788 3b6ce8e9e36c89875e8cf39962fe8920 733bec43a0bf5ade4d97db708e29b185 a5e93c7ff6f4cf893a094f21b0af2747 d3f3523aa6198c328172035f053be461
bitstream.checksumAlgorithm.fl_str_mv	MD5 MD5 MD5 MD5 MD5
repository.name.fl_str_mv	Repositorio Digital Universidad Simón Bolívar
repository.mail.fl_str_mv	repositorio.digital@unisimon.edu.co
_version_	1858228219626389504
spelling	Sanguino-Caneva, Ciro5308a227-65f4-4797-a342-e0dc5f988da1-1Carrillo-Chapman, Carlosbbdba1a3-efd4-4763-b095-06d5c88a48f2-1Luque-Llano, Melissa1297e67a-48a6-47cd-9ed0-fb6e5b40b361-1Bermúdez, Valmore29f9aa18-16a4-4fd3-8ce5-ed94a0b8663a-1Vargas-Manotas, Josédd58499c-4da8-432f-9c36-ba8249458b08-12025-07-07T22:01:55Z2025-07-07T22:01:55Z2023Sanguino-Caneva C, Carrillo-Chapman C, Luque-Llano M et al. Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier [version 1; peer review: 1 approved with reservations, 1 not approved] F1000Research 2023, 12:1254 https://doi.org/10.12688/f1000research.141188.120461402https://hdl.handle.net/20.500.12442/16804https://doi.org/10.12688/f1000research.141188.1https://f1000research.com/articles/12-1254/v1Introduction: Linear scleroderma en coup de sabre is a subtype of scleroderma hallmarked by cutaneous and extracutaneous manifestations in which neurological symptoms can be a predominant feature of this condition. Case presentation: We report a case of a previously healthy 47-year old male who developed neuropsychiatric symptoms and right-sided cephalalgia for two months. Clinical examination revealed a right frontoparietal cutaneous lesion and neurological findings suggesting a medial prefrontal syndrome. The neuroimaging evaluation identified scalp and bone thinning adjacent to the skin lesion and cortical subcortical white matter hyperintensity due to vasogenic oedema at the right frontal and parietal region. A biopsy from the affected area revealed reactive gliosis. Conclusion: To our knowledge, this is the first linear scleroderma en coup de sabre report associated with a neurological involvement typical of a medial prefrontal syndrome. This case highlights the importance of clinical acuity in recognising atypical phenotypes within the spectrum of this uncommon disease.pdfengTaylor and FrancisFacultad de Ciencias de la SaludAttribution-NonCommercial-NoDerivatives 4.0 Internationalhttp://creativecommons.org/licenses/by-nc-nd/4.0/info:eu-repo/semantics/openAccesshttp://purl.org/coar/access_right/c_abf2F1000ResearchVol. 12 No. 1254, (2023)Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrierinfo:eu-repo/semantics/articleArtículo científicohttp://purl.org/coar/version/c_970fb48d4fbd8a85http://purl.org/coar/resource_type/c_2df8fbb1Localized SclerodermaLinear Scleroderma en coup de sabreMorpheaNeurologic involvementNeurologic manifestationsPrefrontal syndromeDenton CP, Khanna D: Systemic sclerosis. Lancet. 2017 Oct 7; 390(10103): 1685–1699. Epub 2017 Apr 13.Rongioletti F, Ferreli C, Atzori L, et al.: Scleroderma with an update about clinico-pathological correlation. G. Ital. Dermatol. Venereol. 2018 Apr; 153(2): 208–215. Epub 2018 Jan 24.Medsger TA Jr, Masi AT: Epidemiology of systemic sclerosis (scleroderma). Ann. Intern. Med. 1971 May; 74(5): 714–721.Zhong L, Pope M, Shen Y, et al.: Prevalence and incidence of systemic sclerosis: A systematic review and meta-analysis. Int. J. Rheum. Dis. 2019 Dec; 22(12): 2096–2107. Epub 2019 Oct 16.Tian J, Kang S, Zhang D, et al.: Global, regional, and national incidence and prevalence of systemic sclerosis. Clin. Immunol. 2023 Mar; 248: 109267. Epub 2023 Feb 15.Careta MF, Romiti R: Localized scleroderma: clinical spectrum and therapeutic update. An. Bras. Dermatol. 2015 Jan-Feb; 90(1): 62–73.Peterson LS, Nelson AM, Su WP, et al.: The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J. Rheumatol. 1997 Jan; 24(1): 73–80.Snarskaya ES, Vasileva KD: Localized scleroderma: actual insights and new biomarkers. Int. J. Dermatol. 2022 Jun; 61(6): 667–674. Epub 2021 Aug 4.Pinho J, Rocha J, Sousa F, et al.: Localized scleroderma en coup de sabre in the Neurology Clinic. Mult. Scler. Relat. Disord. 2016 Jul; 8: 96–98. Epub 2016 May 20.Pierre-Louis M, Sperling LC, Wilke MS, et al.: Distinctive histopathologic findings in linear morphea (en coup de sabre) alopecia. J. Cutan. Pathol. 2013 Jun; 40(6): 580–584. Epub 2013 Mar 18Lis-Święty A, Skrzypek-Salamon A, Ranosz-Janicka I, et al.: Localized scleroderma: clinical and epidemiological features with emphasis on adulthood- versus childhood-onset disease differences. J. Eur. Acad. Dermatol. Venereol. 2017 Oct; 31(10): 1595–1603. Epub 2017 Apr 3.Mertens JS, Seyger MM, Kievit W, et al.: Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric- or adult-onset disease. Br. J. Dermatol. 2015 Mar; 172(3): 722–728. Epub 2015 Feb 8.Mazori DR, Wright NA, Patel M, et al.: Characteristics and treatment of adult-onset linear morphea: A retrospective cohort study of 61 patients at 3 tertiary care centers. J. Am. Acad. Dermatol. 2016 Mar; 74(3): 577–579.Yamasaki R, Yonekawa T, Inamizu S, et al.: A case of overlapping adult-onset linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic involvement. Neuropathology. 2020 Feb; 40(1): 109–115. Epub 2019 Nov 27García Martos Á, González Gómez FJ, Terrancle JI: Stroke-mimic and scleroderma in “coup de sabre”: Case report. Rev Colomb Reumatol Engl Ed. 2021; 28(4): 306–308Amaral TN, Peres FA, Lapa AT, et al.: Neurologic involvement in scleroderma: a systematic review. Semin. Arthritis Rheum. 2013 Dec; 43(3): 335–347. Epub 2013 Jul 1.Gambichler T, Kreuter A, Hoffmann K, et al.: Bilateral linear scleroderma “en coup de sabre” associated with facial atrophy and neurological complications. BMC Dermatol. 2001; 1: 9. Epub 2001 Dec 4.Sarria-Estrada S, Toledo M, Santamarina E, et al.: Esclerosis mesial temporal en un caso de esclerodermia lineal en coup de sabre. Rev. Neurol. 2011; 53(05): 316.Chung MH, Sum J, Morrell MJ, et al.: Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings. Ann. Neurol. 1995 May; 37(5): 679–681.Magro CM, Halteh P, Olson LC, et al.: Linear scleroderma “en coup de sabre” with extensive brain involvement-Clinicopathologic correlations and response to anti-Interleukin-6 therapy. Orphanet J. Rare Dis. 2019 May 16; 14(1): 110.Kister I, Inglese M, Laxer RM, et al.: Neurologic manifestations of localized scleroderma: a case report and literature review. Neurology. 2008 Nov 4; 71(19): 1538–1545.Stone J, Franks AJ, Guthrie JA, et al.: Scleroderma “en coup de sabre”: pathological evidence of intracerebral inflammation. J. Neurol. Neurosurg. Psychiatry. 2001 Mar; 70(3): 382–385.Takahashi T, Asano Y, Oka T, et al.: Scleroderma en coup de sabre with recurrent episodes of brain hemorrhage. J. Dermatol. 2016 Feb; 43(2): 203–206. Epub 2015 Jul 15Corbally CM, Breckenridge A, Jampana R: Imaging and clinical findings in a case of linear scleroderma en coup de sabre. BJR Case Rep. 2016 Nov 2; 2(4): 20150203.Miura S, Someya M, Toyama S, et al.: A case of scleroderma en coup de sabre with ipsilateral hearing loss and aphakia. Eur. J. Dermatol. 2019 Aug 1; 29(4): 423–425.Hock LE, Kontzialis M, Szewka AJ: Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. Neurology. 2016 Oct 18; 87(16): 1741–1742.Rimoin L, Arbiser J: Improvement of “En Coup de Sabre” Morphea and Associated Headaches With Botulinum Toxin Injections. Dermatol. Surg. 2016 Oct; 42(10): 1216–1219.Infante-Valenzuela A, Camara-Lemarroy CR, Delgado-Garcia G, et al.: Steroid-responsive headache in scleroderma en coup de sabre. Acta Neurol. Belg. 2017 Mar; 117(1): 405–407. Epub 2016 Jul 18.Doolittle DA, Lehman VT, Schwartz KM, et al.: CNS imaging findings associated with Parry-Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities. Neuroradiology. 2015 Jan; 57(1): 21–34. Epub 2014 Oct 11.Appenzeller S, Montenegro MA, Dertkigil SS, et al.: Neuroimaging findings in scleroderma en coup de sabre. Neurology. 2004 May 11; 62(9): 1585–1589.Lis-Święty A, Brzezińska-Wcisło L, Arasiewicz H: Neurological abnormalities in localized scleroderma of the face and head: a case series study for evaluation of imaging findings and clinical course. Int. J. Neurosci. 2017 Sep; 127(9): 835–839. Epub 2016 Oct 26Hixon AM, Christensen E, Hamilton R, et al.: Epilepsy in Parry-Romberg syndrome and linear scleroderma en coup de sabre: Case series and systematic review including 140 patients. Epilepsy Behav. 2021 Aug; 121(Pt A): 108068. Epub 2021 May 28.Papara C, De Luca DA, Bieber K, et al.: Morphea: The 2023 update. Front Med (Lausanne). 2023 Feb 13; 10: 1108623.Kurzinski K, Torok KS: Cytokine profiles in localized scleroderma and relationship to clinical features. Cytokine. 2011 Aug; 55(2): 157–164. Epub 2011 May 4.Taniguchi T, Asano Y, Tamaki Z, et al.: Histological features of localized scleroderma 'en coup de sabre': a study of 16 cases. J. Eur. Acad. Dermatol. Venereol. 2014 Dec; 28(12): 1805–1810. Epub 2013 Oct 10.Asano Y, Fujimoto M, Ishikawa O, et al.: Diagnostic criteria, severity classification and guidelines of localized scleroderma. J. Dermatol. 2018 Jul; 45(7): 755–780. Epub 2018 Apr 23Knobler R, Moinzadeh P, Hunzelmann N, et al.: European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J. Eur. Acad. Dermatol. Venereol. 2017 Sep; 31(9): 1401–1424. Epub 2017 Aug 9.Mertens JS, Marsman D, van de Kerkhof PC , et al.: Use of Mycophenolate Mofetil in Patients with Severe Localized Scleroderma Resistant or Intolerant to Methotrexate. Acta Derm. Venereol. 2016 May; 96(4): 510–513.Nguyen K, Atty C, Ree A: Linear scleroderma en coup de sabre presenting with seizures. Radiol Case Rep. 2020 Sep 3; 15(11): 2164–2170.Sede BarranquillaEspecialización en NeurologíaORIGINALPDF.pdfPDF.pdfapplication/pdf801788https://bonga.unisimon.edu.co/bitstreams/2aaba2f9-d690-4df2-8f8c-fa26be22c4fd/download626a1b87b62bc76d308ee3213e6c6788MD51CC-LICENSElicense_rdflicense_rdfapplication/rdf+xml; charset=utf-8899https://bonga.unisimon.edu.co/bitstreams/d3db64e3-9315-44fb-962a-5eb494ccba2e/download3b6ce8e9e36c89875e8cf39962fe8920MD52LICENSElicense.txtlicense.txttext/plain; charset=utf-8381https://bonga.unisimon.edu.co/bitstreams/7ac18877-adae-4c9c-a0df-8851e5d0e306/download733bec43a0bf5ade4d97db708e29b185MD53TEXTPDF.pdf.txtPDF.pdf.txtExtracted texttext/plain49006https://bonga.unisimon.edu.co/bitstreams/476b2dd9-0e48-4ef9-953f-96d43708615f/downloada5e93c7ff6f4cf893a094f21b0af2747MD54THUMBNAILPDF.pdf.jpgPDF.pdf.jpgGenerated Thumbnailimage/jpeg5307https://bonga.unisimon.edu.co/bitstreams/42f9e337-d77d-43a9-a366-595b66f8f00e/downloadd3f3523aa6198c328172035f053be461MD5520.500.12442/16804oai:bonga.unisimon.edu.co:20.500.12442/168042025-10-08 16:08:05.053http://creativecommons.org/licenses/by-nc-nd/4.0/Attribution-NonCommercial-NoDerivatives 4.0 Internationalopen.accesshttps://bonga.unisimon.edu.coRepositorio Digital Universidad Simón Bolívarrepositorio.digital@unisimon.edu.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

Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier

Publicaciones similares