Upsurging cases of en coup de sabre:distinctive clinical manifestations in a series of 41 patients


  • Khalifa E. Sharquie Department of Dermatology, College of Medicine, University of Baghdad. Medical City Teaching Hospital, Iraq
  • Raed I. Jabbar Department of Dermatology, Fallujah Teaching Hospital, Al-Anbar Health Directorate, Anbar, Iraq
  • Inas K. Sharquie Department of Microbiology & Immunology, College of Medicine, University of Baghdad, Baghdad, Iraq


En coupe de sabre, Morphea, Localized scleroderma


Background En coupe de sabre is not uncommon connective tissue disorder of children and adult and often end with marked deep scarring that starts very early and ending with scarring of the skin and scarring alopecia of scalp. Objective The aim of current study is to screen for patients with en coup de sabre among cases with morphea and to evaluate the different clinical and histopathological features of the disease. Patients and methods This is cross sectional descriptive clinical study where all patients with morphea were screened for en coup de sabre and their clinical manifestations including scarring alopecia. Full clinical history and examination were carried for all patients that were seen during the period during from 2014-2023 years. Biopsies for histopathological evaluation were carried out Results A total number of 181 patients with different types of morphea were seen, their ages ranged between 4– 65 years with mean ± SD = 30 ± 15.345. There were141(77.9%) females and 40(22.09%) males with the ratio of 3.52:1. En coup de sabre was detected in 41(22.65%) cases, their ages ranged from 8-32 years with a mean of 20 years,31(75.6%)] cases under 18 years of age, with 30 (73.17%) females,11 (26.82%) males with female to male ratio was 2.72:1. The age onset of the disease was ranged from 4-28 years with mean of 14 years and in 30 (73.17%) of patients the age at onset of the disease was during childhood. Five (12.19 %) cases with face only but no scalp involvement while scarring alopecia was detected in 36(87.8%) of patients. One of the distinctive early features of the en coup de sabre of the face was the presence of rectangular mostly hyperpigmented and sometimes hypopigmented bands. Histopathological assessment showed mild acanthosis with normal dermal-epidermal junction but with pigmented basal layer while the dermis revealed mild inflammatory reaction with collagen deposition in early lesions while complete skin sclerosis was noticed in late lesions.   Conclusion En coup de sabre is a disease of children and young ages and mostly seen among females. There are early and late distinctive clinical and histopathological pictures. It is common cause of scarring of the face and scalp. Early detection of these patients is mandatory to prevent permanent complications.



Careta M, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. Anais Brasileiros de Dermatologia. 2015;90(1): 62-73.

Sharquie KE, Noaimi AA, Eman T. Abdulqader ET,Aljanabi WK. Clinical and histopathological evaluation of pigmented morphea with new insight in relation to etiopathogenesis of the disease. American Journal of Dermatology and Venereology. 2020; 9(2): 21-26.

Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006;18:606-613.

Lausen SR, Daugaard-Jensen J, Lauridsen EF, Kjaer I. Localised scleroderma en coup de sabre affecting the skin, dentition and bone tissue within craniofacial neural crest fields. Clinical and radiographic study of 6 patients. European Archives of Paediatric Dentistry .2019 ;20:339-350.

Graham PM, Gupta N, Altman DA. En coup de sabre. Cutis. 2019 ;103(1):34-36.

Peterson LS, Nelson AM, Su WPD, Mason T, O’Fallon WM, Gabriel SE. The epidemiology of morphea (localised scleroderma in Olmsted County 1960-1993. Journal of Rheumatology .1997; 24(1):73-80.

Niklander S, Marin C, Martinez R, Esguep A. Morphea “en coup de sabre”: an unusual oral presentation. J Clin Exp Dent. 2017 ; 9(2): e315-e318.

Chiu YE, Vora S, Kwon EM, Maheshwari M. A significant proportion of pediatric morphea en coup de sabre and Parry-Romberg syndrome patients have neuroimaging findings. Pediatr. Dermatol. 2012 ;29(6):738-748.

Tolkachjov SN, Patel NG, Tollefson MM. Progressive hemifacial atrophy: a review. Orphanet J Rare Dis. 2015; 10:39.

Kreuter A. Localised scleroderma. Dermatologic therapy. 2012; 25(2):135-147.

Saracino AM, Denton CP, Orteu CH. The molecular pathogenesis of morphea: from genetics to future treatment targets. Br J Dermatol. 2017. 177(1):34-46.

Kurzinski K, Torok KS. Cytokine profiles in localised scleroderma and relationship to clinical features. Cytokine. 2011; 55(2):157-164.

Soma Y, Fujimoto M. Frontoparietal scleroderma (en coup de sabre) following Blaschko’s lines. Journal of the American Academy of Dermatology. 1998; 38(2):366-368.

Wiebel L, Harper JI. Linear morphea follows Blaschko’s lines. Br J Dermatol. 2008; 159:175-181.

Gambichler T, Kreuter A, Hoffman K, Bechara FG, Altmeyer P, Jansen T. Bilateral linear scleroderma “en coup de sabre” associated with facial atrophy and neural complications. BMC Dermatol. 2001; 1:9.

Badea I, Taylor M, Rosenberg A, Foldvari M. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis. Rheumatology. 2009; 48:213-221.

Kreuter A. Localised scleroderma. Dermatologic therapy. 2012 ; 25(2):135-147.

Amaral TN, Neto JFM, Lapa AT, Peres FA, Guirau CR, Appenzeller S. Neurologic Involvement in Scleroderma en Coup de Sabre. Autoimmune Diseases. 2012; Article ID 719685.

Khamaganova I. Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease? Frontiers in Medicine. 2018; 4:2017.

Garofalo Gomez N, Novoa Lopez L, Gomez Garcia AM, Mendez Mendez M. Linear scleroderma en coup de sabre and epilepsy: presentation of a case in a child. Neurologia. 2012; 27(7):449-451.

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):197-199.

Inci R, Inci MF, Ozkan F, Ozturk P. Frontal linear scleroderma en coup de sabre associated with epileptic seizure.BMJ Case Rep.2012 Dec 10 ; 2012:bcr2012007837.

Sehgal VN, Srivastava G, Aggarwal AK, Behl PN, Chou-dhary M, Bajaj P. Localized scleroderma/morphea. Int J Dermatol. 2002; 41:467.

Murray KJ, Laxer RM. Scleroderma in children and adolescents. Rheum Dis Clin North Am. 2002;28:603-24.

Al-Hamamy H, Sharquie KE, Naomi A, Abdulwahhab W. Mycosis Fungoides in Iraqi patients-clinical, histopathological and immunohistochemical study. J Cosmet Dermatol Sci Appl. 2015;5(2):116-24.

Sharquie KE, Al-Jaralla FA, Al-Saadawi AR. Hyperpigmented lichenoid mycosis fungoides as a new variant: clinical and histopathological evaluation. Our Dermatol Online. 2020;11(e):e168.1-e168.5

Sharquie KE, Al-Jaralla FA, Taha S F. Mycosis fungoides in Iraqi population; changing in frequency, demographic and clinical subtypes. J Pak Assoc of Dermatol. 2022;32(3):510-516.

- Sharquie KE, Jabbar RI. Triple therapy of Kaposi’s sarcoma using oral propranolol,oral zinc sulfate and oral acyclovir as new initiative study. J Pak Assoc Dermatol.2023;33(1):72-7.

Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol.2011; 64(2):217–230.

Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) .2006;45:614-20.

Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007;56(2):257-63.




How to Cite

Sharquie KE, Jabbar RI, Sharquie IK. Upsurging cases of en coup de sabre:distinctive clinical manifestations in a series of 41 patients . J Pak Assoc Dermatol [Internet]. 2023Jan.15 [cited 2024Jul.13];34(1):85-91. Available from:



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