Advertisement

Asymptomatic Atrophic Plaque on the Face

  • Yan Ling Kong
  • Emily Yiping Gan
Chapter

Abstract

  • Linear morphea is the most common subtype of localized scleroderma in children

  • When linear morphea affects the face or scalp it is termed as “En coup de sabre” (ECDS)

  • ECDS is commonly associated with neurological complications, with the most common being epilepsy

  • Active inflammatory lesions in ECDS require aggressive management with phototherapy or oral immunosuppressive agents

  • UVA-1 is the preferred phototherapeutic modality and methotrexate is the oral immunosuppressant of choice

Keywords

Linear morphea En coup de sabre UVA Methotrexate 

References

  1. 1.
    Miller K, Lehrhoff S, Fischer M, Meehan S, Latkowski J. Linear morphea of the forehead (en coup de sabre). Dermatol Online J. 2012;18(12):22.PubMedGoogle Scholar
  2. 2.
    Blaszczyk M, Królicki L, Krasu M, Glinska O, Jablonska S. Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol. 2003;30(9):1997–2004.PubMedGoogle Scholar
  3. 3.
    Polcari I, Moon A, Mathes EF, Gilmore ES, Paller AS. Headaches as a presenting symptom of linear morphea en coup de sabre. Pediatrics. 2014;134(6):e1715–9.CrossRefGoogle Scholar
  4. 4.
    Mertens JS, Seyger MM, Kievit W, Hoppenreijs EP, Jansen TL, van de Kerkhof PC, Radstake TR, de Jong EM. Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric- or adult-onset disease. Br J Dermatol. 2015;172(3):722–8.CrossRefGoogle Scholar
  5. 5.
    Zulian F. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology. 2006;45(5):614–20.CrossRefGoogle Scholar
  6. 6.
    Sartori S, Martini G, Calderone M, Patrizi A, Gobbi G, Zulian F. Severe epilepsy preceding by four months the onset of scleroderma en coup de sabre. Clin Exp Rheumatol. 2009;27:64–7.PubMedGoogle Scholar
  7. 7.
    Chiang KL, Chang KP, Wong TT, Hsu TR. Linear scleroderma “En coup De sabre”: initial presentation as intractable partial seizures in a child. Pediatr Neonatol. 2009;50(6):294–8.CrossRefGoogle Scholar
  8. 8.
    Kister I, Inglese M, Laxer RM, Herbert J. Neurologic manifestations of localized scleroderma: a case report and literature review. Neurology. 2008;71(19):1538–45.CrossRefGoogle Scholar
  9. 9.
    Doolittle DA, Lehman VT, Schwartz KM, Wong-Kisiel LC, Lehman JS, Tollefson MM. CNS imaging findings associated with Parry–Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities. Neuroradiology. 2014;57(1):21–34.CrossRefGoogle Scholar
  10. 10.
    Zannin ME, Martini G, Athreya BH, Russo R, Higgins G, Vittadello F, Alpigiani MG, Alessio M, Paradisi M, Woo P, Zulian F. Ocular involvement in children with localised scleroderma: a multi-centre study. Br J Ophthalmol. 2007;91(10):1311–4.CrossRefGoogle Scholar
  11. 11.
    Fett N, Werth VP. Update on morphea. J Am Acad Dermatol. 2011;64(2):217–28.CrossRefGoogle Scholar
  12. 12.
    Taniguchi T, Asano Y, Tamaki Z, Akamata K, Aozasa N, Noda S, Takahashi T, Ichimura Y, Toyama T, Sugita M, Sumida H, Kuwano Y, Miyazaki M, Yanaba K, Sato S. Histological features of localized scleroderma ‘en coup de sabre’: a study of 16 cases. J Eur Acad Dermatol Venereol. 2014;28(12):1805–10.CrossRefGoogle Scholar
  13. 13.
    Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54(3):440–7.CrossRefGoogle Scholar
  14. 14.
    Kerscher M. PUVA bath photochemotherapy for localized scleroderma. Evaluation of 17 consecutive patients. Arch Dermatol. 1996;132(11):1280–2.CrossRefGoogle Scholar
  15. 15.
    El-Mofty M, Mostafa W, El-Darouty M, Bosseila M, Nada H, Yousef R, Esmat S, El-Lawindy M, Assaf M, El-Enani G. Different low doses of broad-band UVA in the treatment of morphea and systemic sclerosis. Photodermatol Photoimmunol Photomed. 2004;20(3):148–56.CrossRefGoogle Scholar
  16. 16.
    El-Mofty M, Mostafa W, Esmat S, Youssef R, Bousseila M, Nagi N, Shaker O, Abouzeid A. Suggested mechanisms of action of UVA phototherapy in morphea: a molecular study. Photodermatol Photoimmunol Photomed. 2004;20(2):93–100.CrossRefGoogle Scholar
  17. 17.
    Rattanakaemakorn P, Jorizzo JL. The efficacy of methotrexate in the treatment of en coup de sabre (linear morphea subtype). J Dermatolog Treat. 2018;29(2):197–9.CrossRefGoogle Scholar
  18. 18.
    Torok KS, Arkachaisri T. Methotrexate and corticosteroids in the treatment of localized scleroderma: a standardized prospective longitudinal single-center study. J Rheumatol. 2012;39(2):286–94.CrossRefGoogle Scholar
  19. 19.
    Li SC, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, Rabinovich CE, Laxer RM, Higgins GC, Ferguson PJ, Lasky A, Baszis K, Becker M, Campillo S, Cartwright V, Cidon M, Inman CJ, Jerath R, O’Neil KM, Vora S, Zeft A, Wallace CA, Ilowite NT, Fuhlbrigge RC. 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):1175–85.Google Scholar
  20. 20.
    Zulian F, Vallongo C, Patrizi A, Belloni-Fortina A, Cutrone M, Alessio M, Martino S, Gerloni V, Vittadello F, Martini G. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea). J Am Acad Dermatol. 2012;67(6):1151–6.CrossRefGoogle Scholar
  21. 21.
    Franco JPDA, Serra MM, Lima RB, D’Acri AM, Martins CJ. Scleroderma en coup de sabre treated with polymethylmethacrylate—case report. An Bras Dermatol. 2016;91(2):209–11.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Yan Ling Kong
    • 1
  • Emily Yiping Gan
    • 2
  1. 1.National Skin CentreSingaporeSingapore
  2. 2.Dermatology ServiceKK Women’s and Children’s HospitalSingaporeSingapore

Personalised recommendations