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En Coup de Sabre

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

Overview

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, 2013 Jun;40(6):580-4.

Complications of En Coup de Sabre

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, 2013 Oct 10.

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, 2013 Mar 18. (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. 01/01/2012.

Support for En Coup de Sabre

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Sclero Forums Discussion on En Coup de Sabre. ISN's Sclero Forums, Chats, Blogs and Videos 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 scleroderma expert to see if it they will recommend it for you. 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.

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

Phototherapy for Specific Sclerosing Skin Conditions. UVA1 is the most studied wavelength in the treatment of morphea, and owing to the sheer amount of evidence it is probably the preferred phototherapeutic modality for this entity. Medscape. 11/14/2012.

Correction of facial linear scleroderma 'coup de sabre' with BoneSource®. Surgical outcome was excellent in cosmetic and functional aspects without any complication. PubMed, J Plast Reconstr Aesthet Surg. [2008] Feb 1.

Autologous fat grafting is a standard method for soft tissue augmentation. Atrophic scars, lipodystrophy and scleroderma en coup de sabre can be treated. PubMed, Hautarzt, [2003] Dec;54(12):1185-9.

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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