En Coup de Sabre

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
En Coup in Overlap
Patient Stories


En Coup de Sabre is a term used when linear scleroderma affects the forehead. It appears as an indented, vertical, colorless, line of skin on the forehead. The indentation looks as though the person may have been struck by a sword.

Linear Scleroderma can appear as a long streak resembling a deep sabre wound — often called "en coup de sabre." NIH.

En Coup de Sabre is a rare, sporadic disease of indeterminate etiology (cause) which is characterized by progressive atrophy and deformity of one side of the face. Seizure disorder is another feature of this disease." Neuroradiology Reading Room V 2.0, Mayo Foundation.

En Coup de Sabre in Overlap

Distinctive histopathologic findings in linear morphea (en coup de sabre) alopecia. Similar follicular remnants have been reported in chemotherapy-induced permanent alopecia but not in alopecia secondary to morphea or other cicatricial alopecias. J Cutan Pathol.

Complications of En Coup de Sabre

Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome (PRS) and En Coup de Sabre (ECDS). Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous findings. PubMed, J Child Neurol, 2020 Oct;35(11):753-762. (Also see Parry Rombergs Syndrome)

Linear scleroderma "en coup de sabre" with extensive brain involvement-Clinicopathologic correlations and response to anti-Interleukin-6 therapy. The interleukin-6 (IL-6) receptor inhibitor tocilizumab was used and the patient improved dramatically, likely reflecting the drug's effect on the replenishment of endothelial progenitor cells. PubMed, Orphanet J Rare Dis, 2019 May 16;14(1):110.

Localised scleroderma en coup de sabre affecting the skin, dentition and bone tissue within craniofacial neural crest fields. SCS conditions seem to extend from the skin in the depth to the sella turcica area within neural crest fields. PubMed, Eur Arch Paediatr Dent, 03/07/2019.

Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre. In addition to the established feature of enophthalmos, the oculometric evidence suggested smaller eye and rectus muscle involvement, interpreted as a secondary negative trophic effect of the overlying skin disorder on eye structures. PubMed, Eye (Lond), 07/04/2018.

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease? There are certain differences in neurological and ophthalmological changes in the diseases. PubMed, Front Med (Lausanne), 2018 Jan 31;4:258.

Histological features of localized scleroderma 'en coup de sabre': a study of 16 cases. The elimination of mutated epidermal cells by immune surveillance contributes to tissue damage and resultant fibrosis in localized scleroderma. PubMed, J Eur Acad Dermatol Venereol.

Distinctive histopathologic findings in linear morphea (en coup de sabre) alopecia. Similar follicular remnants have been reported in chemotherapy-induced permanent alopecia but not in alopecia secondary to morphea or other cicatricial alopecias. Journal of Cutaneous Pathology. (Also see Alopecia)

Diagnosis of En Coup de Sabre

Linear scleroderma "en coup de sabre". Cranial magnetic resonance imaging showed a paraventricular hyperintense signal in the right centrum semiovale. Highbeam Research.

Support for En Coup de Sabre

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Sclero Forums Discussions on En Coup de Sabre. ISN's Sclero Forums is a well-moderated worldwide online support community for people affected by scleroderma (including en coup de sabre) and related illnesses. ISN.

Treatments for En Coup de Sabre

For UVA1 treatment, first contact your doctor to see if it they will recommend it for you. It is not used for lichen sclerosus that is on the genital area. If they recommend and prescribe it for you, try to find UVA1 facilities near you. Some dermatology offices (usually large offices or with major health centers) have UVA1 equipment (not UVB). It may also be possible to buy UVA1 phototherapy equipment for home use. It is manufactured by Daavlin and distributed worldwide. Home users must be monitored by a physician, who will prescribe the necessary dosage and monitor for possible side-effects, such as skin cancer.

Fat Grafting for the Treatment of Scleroderma. Fat grafting in scleroderma patients likely improves skin manifestations by recreating fullness, correcting contour deformities, and improving skin quality. PubMed, Plast Reconstr Surg, 2019 Dec;144(6):1498-1507.

Photos: Before and After of Fat Grafting for En Coup de Sabre submitted by RanchoSteve. Sclero Forums.

A surgical approach to linear scleroderma (LScs) using Medpor® and dermal fat graft. The surgical treatment of LScs using Medpor® and dermal fat graft is an effective treatment modality that can increase patient satisfaction. PubMed, Arch Craniofac Surg, 2019 Apr;20(2):112-115.

Autologous fat grafting: Harvesting techniques. Autologous fat grafting is widely used for soft-tissue augmentation and replacement in reconstructive and aesthetic surgery providing a biocompatible, natural and inexpensive method. PubMed, Ann Med Surg (Lond), 2018 Nov 13;36:212-218.

En Coup de Sabre Patient and Caregiver Stories

Andrea H: Mother of Son with Facial Morphea I am the mother of a six-year-old boy called Alex who is suffering from facial scleroderma…

Audley: Mother of Linear Morphea (en coup de sabre) Patient After one diagnosis of Vitiligo, my motherly intuition told me that the diagnosis was not correct…

Bernarda: Mother of a Localized Morphea en Coup de Sabre Patient She still is not fully conscious of what is happening, while I am devastated…

More En Coup de Sabre Patient and Caregiver Story Listings…

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