| Localized
Scleroderma: Linear |
| 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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| En Coup de Sabre |
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| What is En Coup de Sabre? |
| 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) |
| Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids. Brain magnetic resonance imaging (MRI) showed focal T2 signal increases in the left frontoparietal region directly adjacent to the area of LSCS (Linear scleroderma "en coup de sabre"). Cerebrospinal fluid analysis revealed oligoclonal bands, suggesting an intracerebral inflammatory process. Subsequent pulsed corticosteroid treatment led to a remission of neurological symptoms and to a marked resolution of the MRI lesions. PubMed. J Neurol Neurosurg Psychiatry 2003 May;74(5):661-4. |
| 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) |
| Progressive facial hemiatrophy: a complex disorder not only affecting the face. A report in a monozygotic male twin pair. The occurrence of PFH in one of monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosis of PFH and accurate follow-up is essential to disclose the occurrence of complications. PubMed. Acta Paediatr. 2004 Dec;93(12):1665-9. |
| Neuroimaging findings in scleroderma en coup de sabre. Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. Epilepsy, when present, is related to these brain lesions. Imaging findings and histopathology indicated that the process, most likely focal inflammatory, may be progressive. PubMed. Neurology. 2004 May 11;62(9):1585-9. |
| 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. |
| Linear scleroderma associated with progressive brain atrophy. This study reports one case where neurological involvement may be associated with ipsilateral facial and skull involvement in disorders referred to clinically as LS 'en coup de sabre', and Parry-Romberg syndrome. Neuroimaging studies should be considered in all patients with linear scleroderma, and such studies become necessary when neurological symptoms occur. Brain Dev 2003 Jan;25(1):57-61 PubMed. |
| 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. |
| Expression of transforming growth factor-beta and platelet-derived growth factor in linear scleroderma. These results do not support the hypothesis that the excessive fibroblast cell activity and abnormal collagen deposition observed in LS are associated with downregulation of TGF-beta or PDGF. PubMed. Biomedica. 2003 Dec;23(4):408-15. (Also see: Causes of Scleroderma: Molecular Defect) |
| 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. |
| Treatment of atrophies secondary to trilinear scleroderma en coup de sabre by autologous tissue cocktail injection. The atrophies are left after regression of linear scleroderma "en coup de sabre." They are often irreversible and disfiguring. Several surgical techniques have been attempted to correct atrophies secondary to linear scleroderma en coup de sabre. However, the result often has proved unsatisfactory. Autologous tissue cocktail injection provided excellent cosmetic results. PubMed. Dermatol Surg. 2003 Oct;29(10):1073-5. |
| Scleroderma en coup de sabre with central nervous system and ophthalmologic involvement: treatment of ocular symptoms with interferon gamma. A 12-month course of interferon-gamma stopped progression of visual symptoms caused by orbital fibrous tissue. To our knowledge, this is the first patient with scleroderma en coup de sabre complicated by orbital involvement who was successfully treated with interferon-gamma. PubMed. J Am Acad Dermatol. 2003 Sep;49(3):543-6. |
| 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... |
| (Español/Spanish) Bernarda: Morfea Localizada-Golpe de Sabre Fue un golpe tremendo para mí, ella aún no toma plena conciencia de lo que le pasa, yo estoy destrozada... |
| Bill B: En Coup de Sabre It is now getting near my eyebrow. I am a bit concerned it may begin to encroach on my eye and I am not sure what will happen... |
| Carlos: Linear Sclerodermia (En Coup de Sabre) I have been in treatment, but in my opinion I have not done much better, perhaps it is even worse... |
| (Español/Spanish) Carlos: Esclerodermia Lineal (En Coup de Sabre) Me he sometido a tratamientos y para mi apreciación sigo igual o peor... |
| Carmen: Linear Morphea, En Coup de Sabre I am an otherwise healthy, thirty-eight-year-old female, diagnosed with the en coup de sabre form of linear scleroderma... |
| Cedric: Linear Scleroderma, En Coup de Sabre (South Africa) I became aware of a small brown spot just above my right eye and gradually this patch became bigger and darker... |
| Dora: Linear Scleroderma I was diagnosed with linear scleroderma (en coup de sabre) in Romania 41 years ago... |
| Jane D: Linear Morphea (En Coup de Sabre) In August 2006, I found another dent in my head, but this one was deeper and longer and just to the left of the first one... |
| Joyce R: Scleroderma En Coup de Sabre When I was fifteen years old, somebody at school pointed out to me the fact that there was a white spot in the middle of my forehead, just below the hairline... |
| Laura H: Mother of Son with Linear Morphea What started as a bruise in his forehead, has come down to his nose, and his forehead has sunk a bit... |
| (Español/Spanish) Laura H.: Morfea Lineal Al principio no le dí mucha importancia porque pasaba el tiempo y seguía igual, pero ahora pasado un año todo ha cambiado... |
| Linda E: Mother of En Coup de Sabre and Morphea Patient Five weeks later I noticed that the bruise was still there so we went to a pediatrician who referred us to a dermatologist... |
| Margot: Morphea, Linear and En Coup de Sabre I first went to a medical clinic when I noticed a small brownish mark on my stomach resulting in a doctor telling me I was wearing my jeans too tight! |
| Mary B: Scleroderma En Coup de Sabre I feel like it is tearing my head open. Sometimes I get headaches, and I believe it is because of this illness... |
| (Español/Spanish) Mary B: Esclerodermia en Golpe de Sabre Por el contrario siento que se me está haciendo una enorme brecha en la cabeza. A veces me dan dolores de cabeza y creo que es por causa de esta enfermedad... |
| Tess: Scleroderma En Coup de Sabre I am thirty years old and I have just had reconstructive surgery for en coup de sabre, which is a form of linear scleroderma... |
