En coup de sabre is an unusual variant of localized scleroderma characterized by its distinct location involving the frontoparietal region of the forehead and scalp. The authors describe a rare case of en coup de sabre in a 3-year-old boy whose disease onset was at 12 months of age. This article presents the clinical manifestations of continuous changes in the lesion photographed over time. The clinical presentation, laboratory results, and radiological findings together with a brief discussion of the management of the disease are discussed.
AraneguiBJiménez-ReyesJ. Morphea in childhood: an update. Morfea En la Infancia: Actualización. Actas Dermosifiliogr. 2018;109(4):312–322.
2.
ArkachaisriTPinoS. Localized scleroderma severity index and global assessments: a pilot study of outcome instruments[published correction appears in J Rheumatol. 2009 Sep;36(9):2136]. J Rheumatol. 2008;35(4):650–657.
3.
ArkachaisriTVilaiyukSTorokKSMedsgerTAJr. Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof-of-concept study. Rheumatology (Oxford). 2010;49(2):373–381.
4.
AsanoYFujimotoMIshikawaOSatoSJinninMTakeharaKHasegawaMYamamotoTIhnH. Diagnostic criteria, severity classification and guidelines of localized scleroderma. J Dermatol. 2018;45(7):755–780.
5.
Bielsa MarsolI. Update on the classification and treatment of localized scleroderma. Actas Dermosifiliogr. 2013;104(8):654–666.
6.
BlaszczykMKrólickiLKrasuMGlinskaOJablonskaS. Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol. 2003;30(9):1997–2004.
7.
BoozalisEShahAAWigleyFKangSKwatraSG. Morphea and systemic sclerosis are associated with an increased risk for melanoma and nonmelanoma skin cancer. J Am Acad Dermatol. 2019;80(5):1449–1451.
8.
CaretaMFRomitiR. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol. 2015;90(1):62–73.
9.
ChenJTSchmidDBIsraelJSSiebertJW. A 26-year experience with microsurgical reconstruction of hemifacial atrophy and linear scleroderma. Plast Reconstr Surg. 2018;142(5):1275–1283.
10.
Christen-ZaechSHakimMDAfsarFSPallerAS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol. 2008;59(3):385–396.
11.
ConstantinTFoeldvariIPainCEPálinkásAHögerPMollMNemkovaDWeibelLLaczkovszkiMClementsP, et al.Development of minimum standards of care for juvenile localized scleroderma. Eur J Pediatr. 2018;177(7):961–977.
12.
FettNWerthVP. Update on morphea: Part I. epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol. 2011;64(2):217–230.
13.
Florez-PollackSKunzlerEJacobeHT. Morphea: current concepts. Clin Dermatol. 2018;36(4):475–486.
14.
HerrickALEnnisHBhushanMSilmanAJBaildamEM. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res (Hoboken). 2010;62(2):213–218.
15.
KashemSWCorrellCKVeheRKHobdayPMBinstadtBAMaguinessSM. Inflammatory arthritis in pediatric patients with morphea. J Am Acad Dermatol. 2018;79(1):47–51. e2.
16.
KelseyCETorokKS. The localized scleroderma cutaneous assessment tool: responsiveness to change in a pediatric clinical population. J Am Acad Dermatol. 2013;69(2):214–220.
17.
KimKTSunHChungEH. A surgical approach to linear scleroderma using Medpor and dermal fat graft. Arch Craniofac Surg. 2019;20(2):112–115.
18.
KnoblerRMoinzadehPHunzelmannNKreuterACozzioAMouthonLCutoloMRongiolettiFDentonCPRudnickaL, 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;31(9):1401–1424.
19.
KumarABBlixtEKDrageLAEl-AzharyRAWetterDA. Treatment of morphea with hydroxychloroquine: a retrospective review of 84 patients at Mayo clinic, 1996-2013. J Am Acad Dermatol. 2019;80(6):1658–1663.
LiSCLiXPopeEStewartKHigginsGCRabinovichCEO’NeilKMHainesKALaxerRMPunaroM, et al.New features for measuring disease activity in pediatric localized scleroderma. J Rheumatol. 2018;45(12):1680–1688.
22.
LiSCTorokKSPopeEDedeogluFHongSJacobeHTRabinovichCELaxerRMHigginsGCFergusonPJ, et al.Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res (Hoboken). 2012;64(8):1175–1185.
23.
Lopez PineiroMLeeKPinneyS. A case of linear morphea involving the oral cavity. JAAD Case Rep. 2019;5(2):144–146.
24.
LythgoeHBaildamEBeresfordMWClearyGMcCannLJPainCE. Tocilizumab as a potential therapeutic option for children with severe, refractory juvenile localized scleroderma. Rheumatology (Oxford). 2018;57(2):398–401.
25.
MaleticJTsirkaVIoannidesPKaracostasDTaskosN. Parry-Romberg syndrome associated with localized scleroderma. Case Rep Neurol. 2010;2(2):57–62.
26.
MartiniGFadanelliGAgazziAVittadelloFMeneghelAZulianF. Disease course and long-term outcome of juvenile localized scleroderma: experience from a single pediatric rheumatology centre and literature review. Autoimmun Rev. 2018;17(7):727–734.
27.
MartiniGRamananAVFalciniFGirschickHGoldsmithDPZulianF. Successful treatment of severe or methotrexate-resistant juvenile localized scleroderma with mycophenolate mofetil. Rheumatology (Oxford). 2009;48(11):1410–1413.
28.
MishimaKKitohHMatsushitaMNagataTKamiyaYIshiguroN. Extensive bone lengthening for a patient with linear morphea. Case Rep Orthop. 2018;2018:4535804.
29.
NoakesR. Assessing the response of morphea and limited scleroderma to tranilast: a small prospective study comparing topical corticosteroids to a combination of topical corticosteroids and tranilast. Clin Cosmet Investig Dermatol. 2018;11:321–326.
30.
Peña-RomeroAGGarcía-RomeroMT. Diagnosis and management of linear scleroderma in children. Curr Opin Pediatr. 2019;31(4):482–490.
31.
PetersonLSNelsonAMSuWPMasonTO’FallonWMGabrielSE. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. 1997;24(1):73–80.
32.
PopeEDoriaASTheriaultMMohantaALaxerRM. Topical imiquimod 5% cream for pediatric plaque morphea: a prospective, multiple-baseline, open-label pilot study. Dermatology. 2011;223(4):363–369.
33.
PortaFKaloudiOGarzittoAPrignanoFNacciFFalciniFMatucci CerinicM. High frequency ultrasound can detect improvement of lesions in juvenile localized scleroderma. Mod Rheumatol. 2014;24(5):869–873.
34.
SaracinoAMDentonCPOrteuCH. The molecular pathogenesis of morphoea: from genetics to future treatment targets. Br J Dermatol. 2017;177(1):34–46.
35.
SchochJJSchochBSWerthelJDMcIntoshALDavisDMR. Orthopedic complications of linear morphea: implications for early interdisciplinary care. Pediatr Dermatol. 2018;35(1):43–46.
36.
SeygerMMvan den HoogenFHde BooTde JongEM. Reliability of two methods to assess morphea: skin scoring and the use of a durometer. J Am Acad Dermatol. 1997;37(5 Pt 1):793–796.
37.
SilmanAJanniniSSymmonsDBaconP. An epidemiological study of scleroderma in the West Midlands. Br J Rheumatol. 1988;27(4):286–290.
38.
TorokKS. Pediatric scleroderma: systemic or localized forms. Pediatr Clin North Am. 2012;59(2):381–405.
39.
WeibelLHarperJI. Linear morphoea follows Blaschko’s lines. Br J Dermatol. 2008;159(1):175–181.
40.
Wodok-WieczorekKSalwowskaNSygułaEWodokAWcisło-DziadeckaDBebenekKBergler-CzopBBrzezińska-WcisłoL. The correlation between serum E-selectin levels and soluble interleukin-2 receptors with relation to disease activity in localized scleroderma. Postepy Dermatol Alergol. 2018;35(6):614–619.
41.
ZanninMEMartiniGAthreyaBHRussoRHigginsGVittadelloFAlpigianiMGAlessioMParadisiMWooP, et al.Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol. 2007;91(10):1311–1314.
42.
ZulianFAthreyaBHLaxerRNelsonAMFeitosa de OliveiraSKPunaroMGCutticaRHigginsGCVan Suijlekom-SmitLWMooreTL, et al.Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford).2006;45(5):614–620.
43.
ZulianF. Scleroderma in children. Best Pract Res Clin Rheumatol. 2017;31(4):576–595.
