En coup de sabre meaning. A coup d'état. 3. characteristic Abstract. En coup de sabre presenting as status epilepticus. Neurologic involvement is common and should prompt a comprehensive neurologic workup in patients suspected to have ECDS or PHA. The ings consistent with en coup de sabre morphea, 13 (24. Manifestaciones neurológicas, imágenes y revisión J. May 2, 2007 · Each of these subtypes can involve the face and particularly the orbit. Coup de gras synonyms, Coup de gras pronunciation, Coup de gras translation, English dictionary definition of Coup de gras. In literature, en coup de sabre morphea, and en coup de sabre scleroderma are terms used interchangeably and synonymously. Oct 6, 2023 · Dans cette vidéo j'explique ce qu'est un coup de sabre en maçonnerie après qu'un colonne entre vous me fait la remarque qu'il y en avait sur ma maison. 8%) patients had both. Generalised morphoea. Autologous fat grafting provides a safe and easy approach for the treatment of linear scleroderma "en coups de sabre". Den kliniske præsentation, laboratoriefund, epidemiologi, etiologiske hypoteser og behandlingsmuligheder gennemgås. Eng. J Am Acad Dermatol 2007;56:257–63. Hair is lost over the affected skin, and the skull bone may be shrunk underneath it. Analysis of CSF and pathology obtained at Jun 24, 2013 · Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. ” Approximately one-third of people with linear scleroderma on their head or neck also have Parry-Romberg syndrome. bə(ɹ)/ ~ /ɑ̃ ku də sa. A linear, indurated depression running vertically just to one side of the midline is characteristic of en coup de sabre, a variant of morphea. A sudden appropriation of leadership or power; a takeover: a boardroom coup. Sep 29, 2021 · En coup de sabre (ECDS) is a form of linear scleroderma or morphea that distinctly appears on the forehead and/or frontoparietal scalp. It affects the skin and subcutaneous connective tissues, with possible extension to the underlying musculature, cartilage, and bone and variable A special form, scleroderma en coup de sabre, is a unilateral, linear involvement of the head and face. In most cases, Raynaud's phenomenon is absent. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. En coup de sabre is a rare entity that should be delineated Nov 1, 2005 · En coup de sabre or linear scleroderma has been associated with imaging findings in several reports. Patients often present with neurologic symptoms. Clin Rheumatol. Results: We identified 54 patients who met criteria for inclusion in the study. In most cases, it occurs as a single paramedian line that may be associated with hypoplasia of underlying structures and hemiatrophy of the face. Jul 29, 2011 · A patient with LS en coup de sabre at the Children's Hospital of Alabama was noted to have brain cavernomas by MRI. (2014) Headaches as a presenting symptom of linear morphea en coup de sabre. Objective: To report dermal fat grafting as a novel and effective surgical treatment option in linear scleroderma. The average age of patients was 25 ± 4. 12280 Corpus ID: 12928960; Histological features of localized scleroderma ‘en coup de sabre’: a study of 16 cases @article{Taniguchi2014HistologicalFO, title={Histological features of localized scleroderma ‘en coup de sabre’: a study of 16 cases}, author={Tadatsugu Taniguchi and Yoshihide Asano and Zenshiro Tamaki and Kaname Akamata and Naohiko Aozasa and Shinji Noda Case reports: Here, we report on the cases of 3 patients with LSCS (2 females and 1 male, with a mean age of 40 years). The exact cause is still unknown. May 16, 2019 · The head variant termed “en coup de sabre” (LSES) is characterized by an indurated long streak resembling the deep wound of a sword. They both had an atrophic well-defined linear patch of alopecia on the frontotemporal scalp. The medical records of all patients with diagnoses of en coup de sabre morphea, hemifacial atrophy, or Parry-Romberg syndrome as diagnosed by a dermatologist, rheumatologist, or craniofacial plastic surgeon at the Mayo Clinic from 1984 through 2004 were retrospectively reviewed. A person with a mild case develops a bit of tissue loss. Dec 1, 2014 · DOI: 10. The first time I got it done was tough, as I didn’t get in with a surgeon who was particularly well-versed in the needs of Dec 15, 2012 · Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. Linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. 3 Similarly, not much is known about the efficacy of agents commonly used in treatment of these diseases. Introduction Linear scleroderma “en coup de sabre” (ECDS) is a rare subset of localized scleroderma. En coup de sabre can be very En coup de sabre is a rare subtype of linear scleroderma that is limited to the ipsilateral scalp and face predominately in children and women. It manifests by presence of linear atrophy and/or hardening of the skin, subcutis, occasionally involving muscles and bones. Aug 7, 2023 · Morphea that is the “en coup de sabre” (the blow of a sword) type causes a lesion over the forehead and extends over the scalp leading to alopecia. Linear Scleroderma “en coup de sabre”. Jun 1, 2021 · Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. Imaging findings over a course of 7 years demonstrated waxing and waning signal Oct 20, 2022 · Linear scleroderma , also known as scleroderma en coup de sabre, is a very focal form of scleroderma classically characterised by a linear band of atrophy involving the frontal or frontoparietal scalp and subjacent thinned calvaria associated with ipsilateral focal brain abnormalities. Dec 19, 2022 · The “en coup de sabre” is French for “cut from a sword. En coup de sabre can also be disfiguring. "coup de sabre": examples and translations in context. A. Stone J. 4%) of the 54 patients had bilateral en coup de sabre morphea and/or Parry-Romberg syndrome, whereas the re-maining 50 had en coup de sabre morphea or Parry-Romberg syndrome on one side of the face only. Jun 6, 2023 · The term en coup de sabre morphea refers to a lesion of linear morphea typically located in the frontoparietal scalp and/or the paramedian forehead, often resembling a strike with a sword. Feb 1, 2007 · As en coup de sabre morphea and Parry-Romberg syndrome are relatively rare entities, virtually all information regarding demographics and clinical findings is based on case reports, small case series, and one Internet-based survey. We report a case of a 6-year-old female that presented with En coup de sabre is a rare subtype of linear scleroderma that characteristically affects the skin, underlying muscle, and bone of the frontoparietal region of the face and scalp. 3–4 ), or can be limited to the forehead or scalp, where is it known as en coup de sabre ( Fig. Morphea is differentiated from systemic sclerosis based on the absence of sclerodactyly, Raynaud phenomenon, and nailfold capillary changes. 1111/jdv. It appears as circumscribed linear or triangular induration in the fronto-parietal region, or less frequently, on nose, chin, cheek and neck [ 2 ]. Linear morphea also has a higher rate of relapse compared with other variants. In our patient, gliosis in the area of the basal ganglia was diagnosed with the aid of magnetic resonance imaging. ” She underwent a punch biopsy with resulting Mar 8, 2023 · Ostertag JU et al. A deathblow delivered to end the misery of a mortally wounded victim. b. Although ECDS involves two layers, skin/subcutaneous tissue and bone, the existing literature mainly focuses only on treating the skin/subcutaneous tissue layer. The name comes from its shape, meaning 'like a sword cut'. Abstract. Some people think it may be completely different from linear scleroderma. Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women. The mean (SD) follow-up was 4. Linear scleroderma may coexist with progressive facial What does en coup de sabre‎ mean? en coup de sabre (English) Origin & history From French en coup de sabre‎ (literally "by/in a strike of the sword"), so-called because of its resemblance to the scar of a sabre wound. 4 (23. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Methods: Patients with LScs were evaluated by high-resolution MRI and CT. 8, 9, 10 NOTA CLÍNICA Esclerodermia lineal en coup de sabre. en coup de sabre er en type lineær sklerodermi, der præsenterer på frontal eller frontoparietal hovedbund. The patient was treated with oral steroids and oral methotrexate improving her cutaneous disease. Holland KE, Steffes B, Nocton JJ, et al. Aug 1, 2022 · The mean (SD) age of patients was 34. Pediatrics 134: e1715-1719 . Pronunciation (Brit. Systemic treatment of localized morphea with methotrexate has been reported in a The mean diagnostic delay was 3. It typically presents in the first two decades of life, and may be associated with focal neurological deficits. En coup de sabre: A line of thickened skin forms on the scalp, face, or both, and the tissue beneath disappears. Jan 18, 2023 · The association of morphea en coup de sabre with neurologic manifestations, such as seizures, headaches, facial paralysis and neuropathy, has been reported (1, 83–85). En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. Because the pathogenesis is still unknown, it remains a matter of debate whether cerebral involvement Other involvements that can occur are ocular and neurological; however, it is frequently associated with linear scleroderma, known as en coup de sabre. ” Depending on where this groove is, it can cause serious issues, such as seizures and problems with the teeth, jaw, nose, lips, and eyes. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. Current attempts for cosmetic correction of atrophic lesions must be preceded by an evaluation of disease activity of LM, either by a sufficient clinical assessment or Linear scleroderma en coup de sabre (LCsc) is a rare subset of LS. Rai R et al. ) IPA: /ɒn ˌkuː də ˈseɪ. Jun 12, 2017 · Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. However, there are no case series using methotrexate treatment for En coup de sabre. Nov 1, 2020 · Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. Parry-Romberg syndrome, also known as progressive facial hemiatrophy, is another variant of linear morphea of the head and face, which is characterized by unilateral atrophy of Update on morphea. Linear scleroderma of the face, also known as “en coup de sabre” because it resembles the strike of a sword, 1 affects the frontoparietal area of the head and often involves the ocular adnexa and even the eye. Affected individuals typically have a characteristic atrophic skin lesion involving the fronto-parietal scalp. According to the literature, involvement of the ‘on line’ eye is rare, often just minor, and noticed mainly as an occasional finding. Jahrhundert. coup de sabre synonyms, coup de sabre pronunciation, coup de sabre translation, English dictionary definition of coup de sabre. Sep 29, 2021 · Two patients with localized scleroderma en coup de sabre had a partial response greater than 50%, two had partial response less than or equal to 50%, and one had no response . ECDS has a specific distribution on the frontal scalp and forehead and is usually unilateral. ” This is a French term meaning “cut from a sword,” because of the way it looks. Epilepsy, when present, is related to these brain lesions. In this paper, clinical presentation of LScs and its neurological involvement are described. Besides the lesion in the face or scalp there are also abnormalities of the muscles, fat tissue and skull. Sep 3, 2020 · coup de sabre, is infrequently associated with neurologic symptoms. Sagen om en 54-årig kvinde med en 3 og et halvt års historie om en coup de sabre præsenteres. coups de grâce 1. Oct 20, 2022 · Linear scleroderma , also known as scleroderma en coup de saber, is a very focal form of scleroderma classically characterized by a linear band of atrophy involving the frontal or frontoparietal scalp and subjacent thinned calvaria associated with ipsilateral focal brain abnormalities. Subkortikale Verkalkungen und Hirnatrophie wurden Feb 16, 2024 · Insbesondere beim „en coup de sabre“ und Parry-Romberg-Syndrom, die sich primär im Gesichts- und Kopfbereich manifestieren, wird nicht nur eine MRT-Bildgebung des Kopfes zum Ausschluss einer zentralnervösen Auffälligkeit, sondern auch eine neurologische Vorstellung bei ZNS(Zentralnervensystem)-assoziierten Beschwerden empfohlen [39,40,41 However, it is typical of linear scleroderma en coup de sabre, a rare form of localised scleroderma. En coup de sabre . Erste Beschreibungen von lokalisierter Sklerodermie finden sich bereits im 17. Intracranial calcifications or white matter abnormalities can occur. pl. Iran J Child Neurol. Epub 2020 Jul 18. However, linear morphea, including the en coup de sabre subtype, may remit and reactivate, remain unchanged, or become more extensive with time. This child was one of 4 children with LS en coup de sabre seen, among 59 LS and morphea patients, over 10 years a this institution, and he was the only one of the 4 LS en coup de sabre children imaged with cavernomas by MRI. The case of a 54-year-old woman with a 3 and one-half year history of en coup de sabre is presented. It evened things out, and I got it done about every one to two years. According to the literature, involvement of the ‘on line morphea en coup de sabre: thickened skin causing linear depressions on the forehead only (Find a localized scleroderma specialist at HSS. » View all results. Demographic data, examination findings, and treatments were noted. Systemic treatment of localized morphea with methotrexate has been reported in a Abstract. 9 years. Oct 21, 2021 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. a. In growing children, both linear scleroderma and en coup de sabre can result in distortion of the growing limb or lack of symmetry of both sides of the face. González-Cornejo a LINEAR SCLERODERMA EN COUP DE SABRE. Nov 14, 2023 · This is called “linear morphea en coup de sabre,” French for “the blow of a sword. [3] [4] [5] As with our patient, these are typically ipsilateral to the cutaneous findings Nov 26, 2020 · Definition und Klassifikation. [ 3 ] Jul 4, 2018 · What was known before. In a study with 54 volunteers, about 30% had hemiatrophia faciei progressiva and scléroderma en coup de sabre simultaneously. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle En coup de sabre morphea is a clinical variant of morphea, presenting as a linear depressed, atrophic area in the paramedian forehead or the frontoparietal scalp, resembling a stroke from a sword. Morphea, also known as localized scleroderma, is a rare fibrosing disorder of the skin and underlying tissues. exp. Linear scleroderma may coexist with progressive facial Apr 22, 2024 · Tollefson MM, Witman PM. Systemic treatment of localised morphea with methotrexate has been reported in a few clinical reports. Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp. Discussion: Rheumatology and dermatology were consulted, and the patient was clinically diagnosed with linear scleroderma “en coup de sabre. 2. Although numerous findings on central nervous system (CNS) imaging of PRS and ECS have been reported, the spectrum and frequency of CNS imaging findings and relation to cutaneous and neurologic abnormalities have not been fully characterized. linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and. 1%) had Parry-Romberg syndrome, and 15 (27. Histopathological evidence for gliosis and mixed Background: Linear scleroderma, also known as "en coup de sabre," is a subtype of localized scleroderma that warrants aesthetic correction because it appears on the forehead region in children. Sclerosis in ECDS lesions may invade deeply to involve underlying Dec 13, 2004 · Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. Difficulties can occur in the differential diagnosis of scleroderma en coup de sabre and progressive hemofacial atrophy . Aug 29, 2023 · Noun [ edit] en coup de sabre ( uncountable) ( medicine) A subtype of localised scleroderma that presents with a linear, atrophic depression on the frontoparietal scalp . It can occur as a single lesion, usually on an extremity, can be widespread (Fig. May 11, 2004 · Abstract. Mar 9, 2020 · In en coup de sabre type, the patient may have neurological abnormalities like migraine, trigeminal neuralgia, seizures, behavioral changes, and learning disability. Chef des Stabes. Practical Neurology. A dermatologist can use a filler, such as hyaluronic acid, to restore the face. Parry-Romberg syndrome, also known as progressive facial hemiatrophy, is another variant of linear morphea of the head and face, which is characterized by unilateral atrophy of Jan 27, 2012 · In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Localized scleroderma is a rare disease of unknown etiology. (1994) Bilaterale lineare tempoparietale Sklerodermie en coup de sabre. 20+ year an coup de sabre champion here, meaning I have had a few rounds of filler in my lifetime! I had pretty decent experiences with juvederm. n. The affected region is a Methods: A retrospective review of patients diagnosed with en coup de sabre morphea or Parry-Romberg syndrome at the Mayo Clinic from 1984 to 2004 was conducted. Summer 2015;9(3):62-68. Twenty-six patients (48%) had en coup May 1, 2020 · In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. The clinical presentation, laboratory findings, epidemiology, etiologic hypotheses, and treatment options are reviewed. In comparison with the group of 733 patients with late-onset JLS, CLS presented a significantly more prolonged disease duration at diagnosis and a higher frequency of en coup de sabre subtypes. Apr 1, 2001 · En coup de sabre syndrome is a form of linear scleroderma (LS) involving the skin of the frontoparietal forehead and scalp. The patients with deeper lesions can develop ocular and neurologic symptoms like headaches and seizures and very rarely develop progressive hemifacial atrophy. Pediatr Dermatol 17: 222-224 Jun 1, 2022 · En coup de sabre deformity (ECDS) is a form of localized scleroderma in the frontoparietal region caused by progressive subcutaneous tissue atrophy and bony defect. Although different alterations were observed in the X-ray images, they were all ipsilateral to the coup de sabre. dem Subtyp en coup de sabre und dem nahe verwandten PRS, gehäuft neurologische Symptome wie Migräne und Epilepsie beobachtet werden, soll eine neurologische Untersuchung und zum Ausschluss einer ZNS-Beteiligung eine MRT des Schädels erfolgen 9, 25-29. Note (s) This section has been translated automatically. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Imaging findings over a course of 7 years demonstrated waxing and waning signal ion of “en coup de sabre” ECDS lesions and facial deformities. 8) years, and 101 of 130 (78%) were female. When the face is affected, some strips located on the forehead may be hollow and lead to an appearance termed ""en coup de sabre"". Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. 3 (1. With minimal manipulation of the aspirated fat, combined with overcorrection of the defect, long-term clinically satisfactory results can be obtained. Pathogenesis. … as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. Hautarzt 45: 398-401; Polcari I et al. García-Navarro a, A. Linear scleroderma en coup de sabre with associated neurological abnormalities. This is often associated with neurological symptoms, especially epilepsy. " Mar 6, 2018 · Abstract. Morphea is distinct from systemic sclerosis ( scleroderma ),… percent; Generalized ‒ 8 percent; Linear ‒ 6 percent; En coup de The mean age of onset of “en coup de sabre” is 13 years old, and the majority of patients with linear scleroderma are diagnosed before the age of 18. am Ende des Monats. Idiom: count coup. MATERIALS AND Methods The authors conducted a literature search for reports on surgical interventions for ECDS deformations, in the electronic databases of PubMed, Scopus, and Cochrane Library Databases. Gutiérrez-Aceves a, V. The typical presentation affects frontoparietal region, and the mean age of onset is around 13 years old [ 1 ]. Linear scleroderma “en coup de sabre” is characterized by atrophy and furrowing of the skin of the front parietal region above the level of the eyebrow. (2000) Bilateral en coup de sabre-a rare entity. Linear scleroderma en coup de sabre (LSCS) is the most common form of morphea on the scalp and its trichoscopic features have not been described yet. Jul 4, 2018 · Linear scleroderma of the face (en coup de sabre) is a disfiguring chronic skin disorder, often starting in childhood or adolescence. No side effects or relapses were reported in patients with localized scleroderma en coup de sabre treated with hydroxychloroquine. Due to the rarity of this Mar 8, 2023 · Sclerotherapy that has already occurred usually results in permanent notching with varying degrees of facial disfigurement. Ruiz-Sandoval a, S. a brilliant stratagem; overturn; upset: His win was quite a coup. bɹə/ Adjective en coup de sabre (not Define coup de sabre. Neuere Klassifikationen unterscheiden im Wesentlichen die lineare Sklerodermie mit den Formen linear, en coup de sabre, Parry-Romberg-Syndrom und gemischte Formen. Imaging findings over a course of 7 years demonstrated waxing and waning signal Linear lesions tend to persist for longer than plaque-type lesions, but they often improve over the years. 3–5 ). This is a rare type where there are more plaques spread around different areas of the body. 7) years. doi: 10. There is controversy about whether en coup de sabre is truly a variant of linear En coup de sabre is a type of linear scleroderma that presents on the frontal or frontoparietal scalp. The lesional skin first exhibits contraction and stiffness that lead to characteristic fibrotic plaques with associated linear alopecia. 1 The pansclerotic plaques are ivory in color with hyperpigmented to violaceous borders extending as a paramedian band on the Linear scleroderma. Jul 1, 2021 · En coup de sabre variant of linear morphea (LM) is a rare sclerotic skin disorder characterized by disfiguring linear depression of the frontal or frontoparietal forehead. G. Four (7. We describe a case of. En coup de sabre and Parry–Romberg syndrome are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. Linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the frontoparietal regions of the scalp. Mar 19, 2021 · The en coup de sabre variant of linear scleroderma typically occurs in children. Linear morphea is the most common type of morphea found in school-aged children. Parry-Romberg syndrome. Sep 3, 2020 · Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. Patients with morphea commonly have systemic symptoms, such as malaise Introduction: Parry-Romberg syndrome (PRS) and en coup de sabre (ECS) are variants of morphea. We report 2 adult women with LSCS with distinct dermoscopic findings. Both conditions can be associated with neurologic findings, including Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults. Current treatment recommendations include topical, intralesional, and oral Aug 5, 2019 · Scleroderma en coup de sabre can result in neurological symptoms such as headache or seizures and may even cause structural alterations in the brain. at the end of the month. The differential diagnosis includes, for example, hemiatrophia faciei progressiva. Among certain Native American peoples, a feat of bravery performed in battle, especially the touching of an enemy's body without causing injury. Linear scleroderma of the face (en coup de sabre) is a disfiguring chronic skin disorder, often starting in childhood or adolescence. May 10, 2004 · Objectives: To describe the neuroimaging and clinical findings in patients with localized scleroderma en coup de sabre (LScs). Riestra-Castañeda a, S. RESULTS Twenty-six publications reporting 39 patients were found in the literature. 2020 Dec;39 (12):3885-3886. L. The etiology of the disorder is unknown, although some consider it a variant of morphea (localized scleroderma) and others proposed a malfunction of the sympathetic system as a cause. Romero-Vargas a, G. We present a case of late-onset en coup de sabre of the Jun 17, 2020 · Unlike en coup de sabre, the primary abnormality occurs in the subcutaneous fat, muscle, and bone. ) Systemic scleroderma (systemic sclerosis) Two major forms of systemic sclerosis are recognized: limited systemic sclerosis (also sometimes referred to as CREST syndrome) and diffuse systemic sclerosis. This is a deep form of linear scleroderma affecting the scalp and temple. It typically occurs in children although cases of adult-onset ECDS exist as reported here. Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. Objectives: To describe the neuroimaging and clinical findings in patients with localized scleroderma en coup de sabre (LScs). Linear scleroderma is most common in young girls. The main neurological symptoms were headache and seizures. Cerda-Camacho c, R. Linear scleroderma “en coup de sabre” (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. 1007/s10067-020-05289-9. Nov 27, 2021 · Scleroderma en coup de sabre Scleroderma en coup de sabre is a form of linear scleroderma in which the line of skin thickening occurs on the forehead or elsewhere on the face. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. 7 Dec 12, 2006 · Institutional review board approval was obtained for this study. The ocular abnormalities include absence of eyebrows and eyelashes on the affected areas, enophthalmos, anterior uveitis, and myopathy of the eye muscles. B. En coup de sabre (ECDS) is a rare subtype of linear scleroderma that is limited to the hemiface in a unilateral distribution. Dec 1, 2021 · En coup de sabre deformity (ECDS) is a form of localized scleroderma in the frontoparietal region caused by progressive subcutaneous tissue atrophy and bony defect. We report a unique case of adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda. It can be associated with intracerebral inflammation, epilep-sy, uveitis and other neurologic abnor-malities [25]. The authors performed three-dimensional reconstructions of MRI and CT scans to evaluate brain and bone structures. Jan 27, 2016 · Da bei linearen Formen der ZS, z. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. The disease usually has a benign course Nov 27, 2021 · D) En Coup de Sabre – An unusual form of linear scleroderma on the face or scalp may appear as a white line referred to as “en coup de sabre. Feb 13, 2023 · The association of morphea en coup de sabre with neurologic manifestations, such as seizures, headaches, facial paralysis and neuropathy, has been reported (1, 83–85). Chief of Staff. Although the skin is typically not indurated or bound down, approximately 71% of patients also exhibit cutaneous sclerosis reminiscent of en coup de sabre. Jul 7, 2017 · This is referred to as en coup de sabre because it makes the skin look as though it’s been struck by a sword. Dec 1, 2009 · Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. Bernard-Medina b, F. 2006;6:185-188. su ha ku yt mj iv ds ty ja gd