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En Coup de Sabre

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

What is En Coup de Sabre?

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

A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. A total of 37 of the 51 cases (73%) had abnormal neuroimaging findings, 33 (65%) reported neurological symptoms, and 27 overlapped on both (53%). Seizures and headache were also the most frequently cited symptoms. Pediatr Dermatol. 2012 Nov-Dec; 29(6):738-48.
A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. A total of 37 of the 51 cases (73%) had abnormal neuroimaging findings, 33 (65%) reported neurological symptoms, and 27 overlapped on both (53%). Seizures and headache were also the most frequently cited symptoms. Pediatr Dermatol. 2012 Nov-Dec; 29(6):738-48.
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)
Neurologic Involvement in Scleroderma en Coup de Sabre. Neurologic damage in LScs is frequent and independent of clinical signs and symptoms. Neuroimaging studies should be considered in all LScs patients at the time of the diagnosis. Longitudinal studies should be done to identify progression, even in asymptomatic patients. Autoimmune Diseases, Vol. 2012, Article ID 719685.
Brain cavernomas associated with en coup de sabre linear scleroderma: two case reports. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic resonance imaging. Emily Fain. (7th Space) Pediatric Rheumatology 2011, 9:18.

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

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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