En Coup de Sabre: A Classical Case of Linear Morphea in a Young Woman

Nanda Earlia; Arie Hidayati; Cut Yunita; Fitri Dewi Ismida; Wizurai Wisesa

doi:10.60084/ijcr.v4i1.406

Authors

Nanda Earlia Department of Dermatology Venereology and Aesthetics, Faculty of Medicine, Universitas Syiah Kuala - Dr. Zainoel Abidin, Banda Aceh, Indonesia
Arie Hidayati Department of Dermatology Venereology and Aesthetics, Faculty of Medicine, Universitas Syiah Kuala - Dr. Zainoel Abidin, Banda Aceh, Indonesia
Cut Yunita Department of Dermatology Venereology and Aesthetics, Faculty of Medicine, Universitas Syiah Kuala - Dr. Zainoel Abidin, Banda Aceh, Indonesia
Fitri Dewi Ismida Department of Anatomical Pathology, Faculty of Medicine, Universitas Syiah Kuala - Dr. Zainoel Abidin, Banda Aceh, Aceh, Indonesia
Wizurai Wisesa Department of Dermatology Venereology and Aesthetics, Faculty of Medicine, Universitas Syiah Kuala - Dr. Zainoel Abidin, Banda Aceh, Indonesia

DOI:

https://doi.org/10.60084/ijcr.v4i1.406

Keywords:

Autoimmune skin disorder, Lichen sclerosus, Localized scleroderma , Craniofacial sclerosis, Methotrexate

Abstract

Morphea, or localized scleroderma, is a rare inflammatory disorder characterized by cutaneous sclerosis without systemic involvement. The craniofacial variant, en coup de sabre, is clinically significant due to its potential for deep-tissue extension and neurologic associations. A 22-year-old woman presented with a 10-year history of a progressively widening linear depressed lesion on the forehead. Clinical evaluation showed a well-demarcated atrophic plaque, while laboratory tests, including ANA, were normal. Histopathology revealed thickened collagen bundles with perivascular lymphocytic infiltration, consistent with sclerotic-phase linear morphea. The patient was diagnosed with linear morphea en coup de sabre and treated with methotrexate and corticosteroids, resulting in clinical improvement after 4 weeks. This case highlights the delayed presentation and slow progression of adult-onset craniofacial linear morphea. Despite minimal inflammatory activity, the lesion location carries a risk of deeper involvement, warranting early systemic immunomodulatory therapy. Adult-onset linear morphea en coup de sabre should be recognized in patients with slowly progressive linear atrophic forehead lesions. Early diagnosis and timely systemic treatment are essential to prevent progression and long-term deformity.

Downloads

Download data is not yet available.

References

Florez-Pollack, S., Kunzler, E., and Jacobe, H. T. (2018). Morphea: Current Concepts, Clinics in Dermatology, Vol. 36, No. 4, 475–486. doi:10.1016/j.clindermatol.2018.04.005.
Fett, N., and Werth, V. P. (2011). Update on Morphea, Journal of the American Academy of Dermatology, Vol. 64, No. 2, 217–228. doi:10.1016/j.jaad.2010.05.045.
Papara, C., De Luca, D. A., Bieber, K., Vorobyev, A., and Ludwig, R. J. (2023). Morphea: The 2023 Update, Frontiers in Medicine, Vol. 10. doi:10.3389/fmed.2023.1108623.
Keum, H., Chen, H. W., Haley, R. W., and Jacobe, H. T. (2024). Incidence and Prevalence of Morphea, JAMA Dermatology, Vol. 160, No. 10, 1128. doi:10.1001/jamadermatol.2024.2993.
Hamaguchi, Y., Ueda‐Hayakawa, I., Kaneko, U., Shimizu, M., Miyamae, T., Ishikawa, H., Ae, R., Nakamura, Y., Asano, Y., and Fujimoto, M. (2025). Nationwide Epidemiological and Clinical Survey of Juvenile‐onset Morphea in Japan, The Journal of Dermatology, Vol. 52, No. 5, 860–871. doi:10.1111/1346-8138.17684.
Saleem, A. N., Krupanandan, R. K., Kapalavai, S. K., Ramachandran, B., Kathirvelu, G., Ramesh, V., Janarthanan, M., Rabia, S., and Ramamoorthy, R. (2024). Linear Scleroderma of Scalp “En Coup de Sabre” in a Child Presenting with Rapidly Deteriorating Acute Encephalopathy, Indian Pediatrics Case Reports, Vol. 4, No. 3, 168–172. doi:10.4103/ipcares.ipcares_38_24.
Mahmood, F., Nguyen, A., Muntyanu, A., Jfri, A., McCuaig, C., Chédeville, G., Piram, M., and Netchiporouk, E. (2022). Prevalence and Incidence of Localized Scleroderma: A Qualitative Systematic Review, Journal of Cutaneous Medicine and Surgery, Vol. 26, No. 6, 632–633. doi:10.1177/12034754221129876.
Mercuri, S. R., Di Nicola, M. R., Bianchi, V. G., and Paolino, G. (2023). Adult-Onset Linear Morphea (En Coupe de Sabre) of the Face Successfully Treated with Photoactivated Low-Temperature Platelet-Rich Plasma: A Valid Therapeutic Option, Medicina, Vol. 59, No. 6, 1114. doi:10.3390/medicina59061114.
Gorolay, V. V., Fisicaro, R., Tsui, B., Tran, N.-A., Eltawil, Y., Glastonbury, C., and Wu, X. (Cynthia). (2025). Neuroimaging and Clinical Features of Parry-Romberg Syndrome and Linear Morphea En-Coup-de-Sabre in a Large Case Series, Academic Radiology, Vol. 32, No. 7, 4154–4163. doi:10.1016/j.acra.2025.02.030.
Kok, L. T., Malik, M., Fayers, T., Sorkou, K., Issa, A., and Ezra, D. G. (2025). Linear Scleroderma En Coup de Sabre: A Case Series With Eyelid Involvement and Management, Ophthalmic Plastic & Reconstructive Surgery, Vol. 41, No. 3, e80–e85. doi:10.1097/IOP.0000000000002843.
Mertens, J. S., Seyger, M. M. B., Thurlings, R. M., Radstake, T. R. D. J., and de Jong, E. M. G. J. (2017). Morphea and Eosinophilic Fasciitis: An Update, American Journal of Clinical Dermatology, Vol. 18, No. 4, 491–512. doi:10.1007/s40257-017-0269-x.
Careta, M. F., and Romiti, R. (2015). Localized Scleroderma: Clinical Spectrum and Therapeutic Update, Anais Brasileiros de Dermatologia, Vol. 90, No. 1, 62–73. doi:10.1590/abd1806-4841.20152890.
Griffiths, C. E. ., Barker, J., Bleiker, T., Chalmers, R., and Creamer, D. (2024). Rook’s Textbook of Dermatology, (C. E. . Griffiths, J. Barker, T. Bleiker, R. Chalmers, & D. Creamer, Eds.) (tenth edit.), Wiley Blackwell.
Torok, K. S., and Arkachaisri, T. (2012). Methotrexate and Corticosteroids in the Treatment of Localized Scleroderma: A Standardized Prospective Longitudinal Single-Center Study, The Journal of Rheumatology, Vol. 39, No. 2, 286–294. doi:10.3899/jrheum.110210.
Amaral, T. N., Marques Neto, J. F., Lapa, A. T., Peres, F. A., Guirau, C. R., and Appenzeller, S. (2012). Neurologic Involvement in Scleroderma En Coup de Sabre, Autoimmune Diseases, Vol. 2012, 1–6. doi:10.1155/2012/719685.
William, J., Elston, D. M., Treat, J. R., Rosenbach, M. A., and Neuhaus, I. M. (2020). Andrew’s Diseases of the Skin Clinical Dermatology (Thirteenth.), Elsevier.
Skrzypek-Salamon, A., Lis-Święty, A., Ranosz-Janicka, I., and Brzezińska-Wcisło, L. (2018). Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) Adapted for Use in Adult Patients: Report from an Initial Validation Study, Health and Quality of Life Outcomes, Vol. 16, No. 1, 185. doi:10.1186/s12955-018-1010-z.
Kreuter, A., Krieg, T., Worm, M., Wenzel, J., Moinzadeh, P., Kuhn, A., Aberer, E., Scharffetter‐Kochanek, K., Horneff, G., Reil, E., Weberschock, T., and Hunzelmann, N. (2016). German Guidelines for the Diagnosis and Therapy of Localized Scleroderma, JDDG: Journal Der Deutschen Dermatologischen Gesellschaft, Vol. 14, No. 2, 199–216. doi:10.1111/ddg.12724.
Kang, S., Amagai, M., Bruckner, A. L., Enk, A. H., Margolis, D. J., McMichael, A. J., and Orringer, J. S. (2019). Fitzpatrick’s Dermatology 9th Edition, McGraw-Hill Education.