| Localized Scleroderma: Linear |
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| En Coup de Sabre |
| This page was written by Shelley Ensz, and medically edited by Dolores Vázquez-Abad, M.D.. The sections on En Coup de Sabre and Parry-Rombergs have not been medically edited yet. See Disclaimer. |
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| 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 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. |
| Linear scleroderma en coup de sabre. Neurological symptoms, images and review. When it affects the central nervous system, the clinical and radiological presentation of LSCS is heterogeneous. Both the imaging studies carried out during the clinical control and the histopathological findings suggest a focal inflammatory process that can be progressive. PubMed. Rev Neurol. 2005 Nov 1-15;41(9):534-7. |
| Clinical and serological characteristics of progressive facial hemiatrophy (PFH): a case series of 12 patients. The reported cases suggest a close relationship between PFH and LSCS (linear scleroderma en coup de sabre). PFH might appear as two different subtypes. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. PubMed. J Am Acad Dermatol. 2006 Feb;54(2):227-33. |
| En Coup de Sabre in overlap |
| En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. En coup de sabre morphea and Parry-Romberg syndrome frequently coexist and are likely both variants of morphea. Bilateral disease is more common than previously reported. The efficacy of antimalarials and methotrexate in the treatment of these diseases remains unclear. PubMed. J Am Acad Dermatol. 2007 Feb;56(2):257-63. (Also see: Parry-Romberg Syndrome ) |
| Complications of En Coup de Sabre |
| En Coup de Sabre. When the face or scalp is affected with linear morphea, the involvement is often compared to a stroke from a sword (sabre), usually on the paramedian forehead. En coup de sabre is almost always unilateral, but rare bilateral cases have been reported. Loss of the eyebrow or eyelashes, ptosis, pseudo-oculomotor palsy, uveitis, asymmetry of the tongue, altered dentition, and dental caries may also occur. Mayo Clinic. (Also see: Scleroderma Dental Involvement) |
| Correspondence to: Linear Scleroderma en Coup de Sabre and Brain Calcification: Is There a Pathogenic Relationship? We describe a child with similar clinical findings. A 6-year-old girl was admitted to our clinic due to skin discoloration and depression on her right forehead and chin. ÖZGÜR KASAPÇOPUR, MD. J Rheumatol NO. 12 DECEMBER 2003;30:2724-25. |
| Progressive Facial Hemiatrophy (PFH): Central Nervous System Involvement and Relationship with Scleroderma en Coup de Sabre. Our results suggest frequent CNS involvement in PFH cases, regardless of the time of presentation of cutaneous indurations, with or without coexistent plaques of localized scleroderma in other locations. This indicates a close relationship between PFH and scleroderma en coup de sabre. J Rheumatol. Vol. 30: No. 9 September 2003;30:1997-2004. |
| Linear Scleroderma en Coup de Sabre and Brain Calcification: Is There a Pathogenic Relationship? We describe a female patient with linear scleroderma en coup de sabre and a longstanding clinical history of tonic and clonic convulsions. Radiographic study showed extensive cerebral calcifications in the right occipital hemisphere, homolateral to the involved side of her face. Brain imaging studies should be routinely performed in scleroderma patients exhibiting neurological manifestations, especially seizure disorder. Diana Elsa Flores-Alvarado. J Rheumatol 2003;30:193-5. |
| Diagnosis of En Coup de Sabre |
| Neuroimaging Findings in Parry Romberg Syndrome and Scleroderma En Coup Sabre. In both syndromes there were signs of cortical abnormalities underneath the skin lesions. Whether this represents a cortical dysgenesis or a progressive degenerative/inflammatory lesion remains to be determined. Follow up brain imaging multicentric collaborative studies may clarify this issue. S. Appenzeller. AB0326 EULAR 2003. |
| Treatments for En Coup de Sabre |
| Correction of facial linear scleroderma 'coup de sabre' with BoneSource®. Surgical outcome was excellent in cosmetic and functional aspects without any complication. The patient was followed for 36 months after the operation; the defect was satisfactorily corrected and no postoperative problems have been found. (PubMed) J Plast Reconstr Aesthet Surg. 2008 Feb 1. |
| Familial linear scleroderma (en coup de sabre) responsive to antimalarials and narrowband ultraviolet B therapy. A 32-year-old woman and her 35-year-old sister presented with plaques of scleroderma en coup de sabre. Treatment of the younger sister with antimalarials and narrow-band ultraviolet B (NB-UVB) phototherapy slowed disease progression and reversed hair loss. PubMed. Dermatol Online J. 2007 Jan 27;13(1):11. |
| 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. |
| Repair of a large "coup de sabre" with soft-tissue expansion and artificial bone graft. This is the first reported case in which a deformity of scleroderma was treated with a combination of an expanded skin flap and a hydroxyapatite implant. Ann Plast Surg. 1999 Feb;42(2):207-10. |
| 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... |
