Is en coup de sabre dangerous

 

Is en coup de sabre dangerous. The patients with deeper lesions can develop ocular and neurologic symptoms like headaches and seizures and very rarely develop progressive hemifacial atrophy. In a study with 54 volunteers, about 30% had hemiatrophia faciei progressiva and scléroderma en coup de sabre simultaneously. It affects the skin and subcutaneous connective tissues, with possible extension to the underlying musculature, cartilage, and bone and variable 2. This is a sclerosing connective-tissue disease of the skin that may also Dec 21, 2017 · I'm sorry to hear about your daughter. There is controversy about whether en coup de sabre is truly a variant of linear Feb 1, 2017 · Abstract. PMID: 29572218. 1111/jdv. En Coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. J Am Acad Dermatol 2007;56:257-263. Apr 14, 2022 · Linear scleroderma en coup de sabre (LSCS) is a variant of localized scleroderma associated with band-like fibrotic lesions in the frontoparietal area. They both had an atrophic well-defined linear patch of alopecia on the frontotemporal scalp. My grandson was diagnosed with linear scleroderma with en coup de sabre last year when he had just turned 7. Linear scleroderma “en coup de sabre” (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. Methods: Patients with LScs were evaluated by high-resolution MRI and CT. Dec 1, 2009 · Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. It can occur as a single lesion, usually on an extremity, can be widespread (Fig. Here, we … Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. 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. In our patient, gliosis in the area of the basal ganglia was diagnosed with the aid of magnetic resonance imaging. MRI orbit was suggestive of the bulky MR and the inferior rectus muscle. Frontoparietal scleroderma (en coup de sabre) following Blaschko’s lines. 8%) patients had both. Octroie 1 point de combo. Headaches, uveitis and episcleritis are also sometimes present. Aug 29, 2023 · The clinical picture was suggestive of a combination of scleroderma en coup de sabre and facial hemiatrophy (Parry-Romberg syndrome). Torok at Children's Hospital in Pittsburgh. 9 Eur J Intern Med. The average age of patients was 25 ± 4. Parry-Romberg syndrome and morphoea en coup de sabre. characteristic Oct 1, 1990 · Scleroderma “en coup de sabre” With Epilepsy and Uveitis Successfully Treated With Tocilizumab Reumatología Clínica (English Edition), Volume 16, Issue 5, Part 1, 2020, pp. A person with a mild case develops a bit of tissue loss. It appears as circumscribed linear or triangular induration in the fronto-parietal region, or less frequently, on nose, chin, cheek and neck [ 2 ]. En coup de sabre (ECDS) is a rare form of localized scleroderma that typically manifests in children and women. Dermatol Reports 2011; 3: e27. ings consistent with en coup de sabre morphea, 13 (24. 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. En coup de sabre can also be disfiguring. She was a known case of linear morphea diagnosed by scalp biopsy, elsewhere. 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 . This case describes a 36-year-old female who presented with a history of en coup de sabre with En coup de sabre is a type of linear scleroderma that presents on the frontal or frontoparietal scalp. Linear scleroderma may coexist with progressive facial En coup de sabre presenting as status epilepticus. Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. Skin lesions are usually ivory-colored, may have violaceous borders, and are often associated with Apr 25, 2023 · Objective:To describe the neurologic complications and imaging findings in a patient with linear scleroderma en coup de sabre (LScs) and associated Parry-Romberg syndrome. Many patients have comorbid central nervous system involvement in addition to the Jan 27, 2012 · In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. 1684/ejd. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Morphoea en coup de sabre is a variant of linear morphoea (a localised form of scleroderma) restricted to the frontoparietal region (forehead). The patient was diagnosed with linear scleroderma “en coup de sabre” (LSECDS). frontoparietal scalp and/or the paramedian forehead, often resembling a strike with a sword. Clin Rheumatol. The affected region is a depression that may be Dec 1, 2021 · En coup de sabre (ECDS) deformity is a rare presentation of localized scleroderma with cutaneous and extracutaneous manifestations. 5-year history of linear 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. , Witman, P. 3. 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. Here, we report one patient with typical linear scleroderma “en coup de sabre” (LSCS). 3–5 ). The clinical presentation, laboratory findings, epidemiology, etiologic hypotheses, and treatment options are reviewed. Affiliation. Lesions form depressed grooves that resemble a sword wound or the stroke of a saber. 8 Tollefson, M. Although it is 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. A subtype of morphea, Scleroderma En Coup De Sabre (ECDS), affects the face and/or scalp with a linear induration of the skin and sometimes involvement of the underlying muscle and bone. 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. Linear scleroderma. We report a case of a 6-year-old female that presented with a linear, hyperpigmented scar on her left forehead extending to her scalp with resultant alopecia and discoloration in the affected area. The disease may be associated with facial hemiatrophy. Indian J Dermatol 2015; 60: 420. A Case of Linear Scleroderma En Coup de Sabre Accompanied with Migraine with Visual Aura and Idiopathic Intracranial Hypertension Indian J Dermatol . Lesions generally occur unilaterally in the form of a Blashkoid distribution, potentially involving deeper neurologic, ocular or oro-dental tissues ( 5 ). Linear morphea occurs in a line usually on the head, arm or leg. Mar 8, 2023 · Sclerotherapy that has already occurred usually results in permanent notching with varying degrees of facial disfigurement. A dermatologist can use a filler, such as hyaluronic acid, to restore the face. 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. In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. He also has a lot of leg and hand pain. Mar 19, 2021 · It can be subdivided into linear, circumscribed (plaque), generalized, pansclerotic, and mixed subtypes. Overall, the clinical and histological findings pointed to linear circumscribed scleroderma (scleroderma en coup de sabre). I ended up getting a fat graft 5 years ago and it made a much bigger difference. The differential diagnosis includes, for example, hemiatrophia faciei progressiva. We diagnosed her as a case of linear scleroderma en coup de sabre (LSCS). linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and. Nov 27, 2021 · 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. Linear scleroderma is most common in young girls. Apr 30, 2018 · Although scleroderma en coup de sabre is a form of localized scleroderma, it is not uncommon to find systemic manifestations in patients with this condition, and it is estimated that roughly one quarter of patients with localized scleroderma will have extracutaneous manifestations. 4103/ijd. In most cases, it occurs as a single paramedian line that may be associated with hypoplasia of underlying structures and hemiatrophy of the face. The predominant localization is on scalp to forehead (6, 21–23). My en coup de sabre scar is pretty deep, I started getting filler for it about 6-7 years ago. 3–4 ), or can be limited to the forehead or scalp, where is it known as en coup de sabre ( Fig. The Key Words: Linear scleroderma, en coup de sabre, epilepsy Abstract Observation: Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. I would go in once every 6-9 months for additional filler/touch ups. ECDS develops through the stages of proliferation, inflammation phase, atrophy, and residual deformity. A coup d'état. En coup de sabre (ECDS) is a form of linear scleroderma or morphea that distinctly appears on the forehead and/or frontoparietal scalp. Objective: To report dermal fat grafting as a novel and effective surgical treatment option in linear scleroderma. Morphea is distinct from systemic sclerosis ( scleroderma ),… percent; Generalized ‒ 8 percent; Linear ‒ 6 percent; En coup de 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. Background:LScs is a rare subset of localized scleroderma. Linear scleroderma may coexist with progressive facial 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. The filler by itself helped, but didn't fill the scar completely. We describe a case of. 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. 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. … as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. BackgroundLinear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face Abstract. It is an autoimmune phenomenon, and the hallmark sign is a cutaneous lesion in the frontoparietal region. We report 2 adult women with LSCS with distinct dermoscopic findings. Extragenital lichen sclerosus et atrophicus. Aug 6, 2011 · In a study conducted Tollefson and Witman 22, among 54 patients diagnosed with PRS or en coup de sabre, only one patient had en coup de sabre on one side and PRS on the other. We report a case of LSA with frontoparietal distribution, mimicking scleroderma en coup de sabre, causing scarring alopecia. Mar 27, 2010 · En coup de sabre is a linear scleroderma that presents on the frontal or frontoparietal scalp. When it occurs as alopecia of the scalp, eyebrow, eyelashes, or forehead, it is termed linear scleroderma en coup de sabre (LSCS). 1 Systemic involvement in LS is extremely rare. It manifests by presence of linear atrophy and/or hardening of the skin, subcutis, occasionally involving muscles and bones. Chaque charge augmenté également les dégâts de Coup de sabre et Attaque pernicieuse de 20 %. Initially Jul 6, 2023 · Abstract. ion of “en coup de sabre” ECDS lesions and facial deformities. It never migrated for me. I was wondering if it could be en coup de sabre? Anyone ever see this? Also, what is en coup de sabre exactly? Is it dangerous? Will it get worse? Do people get treatment? I went on google and got really worried. En coup de sabre: A line of thickened skin forms on the scalp, face, or both, and the tissue beneath disappears. 2019 Dec 1;29 (6):661-662. 1007/s10067-020-05289-9. Only a few bilateral cases have been reported to date. doi: 10. J Am Acad Dermatol 1998;24:467-8. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. We present a case of late-onset en coup de sabre of the Objective: Since en coup de sabre (ECDS) is a certain subset of localized scleroderma with a relatively uniform clinical manifestation, we focused on this disease subset and evaluated its histopathological features. 1136/archdischild-2018-314900. A sudden appropriation of leadership or power; a takeover: a boardroom coup. We describe an unusual case of LSC. 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. The name ‘en coup de sabre’ (the blow of a sword) derives from the characteristic scar that indents the skin of the scalp and the underlying bone. Methods: A total of 16 patients with ECDS were retrospectively evaluated on the basis of clinical and histological findings. Mar 13, 2019 · Facial morphea linear lesions (eg, en coup de sabre lesions) may be associated with behavioral changes, learning disabilities and epilepsy. Linear scleroderma may coexist with progressive facial 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. May 10, 2004 · Objectives: To describe the neuroimaging and clinical findings in patients with localized scleroderma en coup de sabre (LScs). Linear morphea en coup de sabre is a localized form of morphea that presents as paramedian face or frontal scalp depression. 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 Linear scleroderma (LS) is a variant of localized scleroderma most commonly seen in the lower limbs and to a lesser extent in the arms and face. She is caring, and very thorough. The histopathology specimens and neuroradiology investigations reaffirm the clinical impression that inflammation is not solely confined to the skin in this form of linear scleroderma. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution on the frontoparietal scalp with surrounding scarring alopecia. Abstract. En coup de sabre (ECDS) is a rare subtype of linear scleroderma that is limited to the hemiface in a unilateral distribution. The detection … The "en coup de sabre" subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. ijd_992_22. Apr 14, 2022 · Linear scleroderma is a localized scleroderma of unknown etiology characterized by band-like sclerotic lesions of the skin and underlying tissues. Cutaneous manifestations of ECDS are progressive. Dec 4, 2001 · The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome) and L SCS actually represent different spectra of the same disease. A am a 23F. 1 Linear scleroderma commonly affects the limbs or the face (en coup de sabre (ECDS) or progressive facial hemiatrophy) and may be associated with extracutaneous complications. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic resonance imaging. 6 A study done in 2007 found that the scleroderma en coup de Oct 21, 2021 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Mar 21, 2024 · En coup de sabre is a rare form of linear scleroderma that affects the head, especially the forehead. 1 LSCS can cause significant functional disability and cosmetic Sep 3, 2020 · coup de sabre, is infrequently associated with neurologic symptoms. ” Approximately one-third of people with linear scleroderma on their head or neck also have Parry-Romberg syndrome. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. 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. The lesion improved with 2 cycles of antibiotic May 16, 2019 · Linear scleroderma "en coup de sabre" (LSES) variant is a cephalic subtype of localized scleroderma that can be associated with extracutaneous stigmata, such as epilepsy, dementia syndromes, as well as focal central nervous system neurologic deficits. Note (s) This section has been translated automatically. Coup de sabre bénéficie A case of an elderly woman who presented with an asymptomatic alopecic plaque consistent with En coup de sabre is reported, which is a rare entity that should be delineated from Parry-Romberg syndrome. Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis. Among certain Native American peoples, a feat of bravery performed in battle, especially the touching of an enemy's body without causing injury. Jun 14, 2023 · En coup de sabre is a rare subtype of morphea. b. 7 Dec 19, 2022 · The “en coup de sabre” is French for “cut from a sword. The lesional skin first exhibits contraction and stiffness that lead to characteristic fibrotic plaques with associated linear alopecia. 2023 May-Jun;68(3):346-348. Faced with no gold standard for treatment of LSECDS with severe extracutaneous manifestations, the case was intensively discussed within an interdisciplinary team setting and Sep 3, 2020 · Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. This indicates a close relationship between PFH and scleroderma en coup de sabre. ) 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. 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. 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 Mar 6, 2018 · Abstract. Morphea is an idiopathic sclerosing disorder of the skin and underlying tissues. The authors performed three-dimensional reconstructions of MRI and CT scans to evaluate brain and bone structures. 1,2 The term “en coup de sabre” is used for linear scleroderma located in the temporoparietal region that produces sharp bordered furrowing with alopecia when extends to the scalp. When involving the scalp and face, linear morphea has been referred to as morphea en coup d’sabre (linear scleroderma can produce a scarred appearance on the face and scalp similar to what might result from the strike of a sword). MR recession along with partial loop Vous obtenez la technique Coup de sabre :Taille sauvagement un ennemi en infligeant 100 % des dégâts de l’arme et fait saigner la cible pour (Attack power * 3 / 100) points de dégâts toutes les 2 s pendant 12 sec, cumulable jusqu’à 5 fois. Four (7. Epub 2020 Jul 18. The case of a 54-year-old woman with a 3 and one-half year history of en coup de sabre is presented. Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women. A case of scleroderma 'en coup de sabre' presenting with uveitis, predominantly posterior, and intractable grand mal seizures is described. Jun 12, 2020 · We present a rare case of a 4-year-old girl suffering from a sclerotic lesion on the forehead as well as uveitis and epileptic seizures. We present a 26-year-old Caucasian female with a 1. We see Dr. It is often associated with the form of linear localized scleroderma (LS) on the scalp and forehead termed En Coup de Sabre (ECDS). Distinction between Parry-Romberg syndrome and linear scleroderma with facial atrophy can be quite difficult and it has been postulated that en coup de sabre type may actually be a forme fruste of progressive facial hemiatrophy. Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. 2009 May;20(3):331-6. Case 1 was a 37-year-old woman who had right hemifacial atrophy with unilateral en coup de sabre for seven years. The lesion improved with 2 cycles of antibiotic Apr 1, 2022 · Il s’agit d’une patiente de 32 ans, ayant pour antécédent une sclérodermie localisée « en coup de sabre » frontale gauche (SLcs) depuis l’âge de 14 ans, ayant présenté des vertiges associés à des céphalées. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution Jan 30, 2020 · Gubertini N, Bonin S, Trevisan G. 1%) had Parry-Romberg syndrome, and 15 (27. Among the cases of PRS available in the Korean medical literature, only four cases were accompanied by en coup de sabre, and no case showed bilateral involvement 24-27. We report a case of LSCS in a woman who presented with progressive mild hyperchromia on the right side of her forehead, with dermal atrophy and hair and eyebrow loss. Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults. Apr 25, 2013 · En coup de sabre is a rare subtype of linear scleroderma characterized by band-like areas of skin sclerosis affecting the frontoparietal region of the scalp, with subsequent involutional atrophy of the skin, subcutaneous tissues, muscle, and bone. 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. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. 2020 Dec;39 (12):3885-3886. L’examen montrait une paralysie du III gauche sans diplopie associée. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic lesions over the forehead with hair loss on the scalp. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. Imaging findings over a course of 7 years demonstrated waxing and waning signal 7 Soma, Y. No side effects or relapses were reported in patients with localized scleroderma en coup de sabre treated with hydroxychloroquine. Idiom: count coup. En coup de sabre is usually a linear unilateral depression on the frontoparietal scalp or paramedian forehead. Mar 1, 2007 · En coup de sabre (ECDS, "from a blow of the sword") is a type of linear scleroderma which begins as a line of cutaneous inflammation and develops into a line of fibrosis (as is the usual pattern Dec 1, 2014 · DOI: 10. Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. 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. However, there are no case series using methotrexate treatment for En coup de sabre. Imaging findings over a course of 7 years demonstrated waxing and waning signal Dec 1, 2019 · Linear facial lichen sclerosus mimicking scleroderma 'en coup de sabre'. Jul 29, 2011 · When it occurs on the scalp or forehead, it is termed "en coup de sabre". The case was associated with Borrelia infection. Dec 4, 2001 · Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. Once thought to be an This is a report of the author's experiences with PRS accompanying en coup de sabre, and a review of the ongoing considerable debate associated with these two entities. Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. 1 ECDS typically occurs in children and is characterized by a Nov 4, 2019 · Linear LS lesions located on the scalp and forehead are commonly termed en coup de sabre (ECDS) for their resemblance to scars caused by a sword strike to the head. While the pathophysiology of cutaneous linear sc … Aug 1, 2022 · The term en coup de sabre morphea refers to a lesion of linear morphea typically located in the. Jun 24, 2013 · Scleroderma "en coup de sabre" Face: Unilateral atrophy: Unilateral, frontoparietal sclerotic band Minimal or absent induration or previous inflammation: Usually preceded by skin induration Cutaneous atrophy (normal hair and absent sclerosis) Usually does not extend below the eyebrow Aug 3, 2011 · Lichen sclerosus et atrophicans (LSA) is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. 3664. 2019. Analysis of CSF and pathology obtained at Sep 28, 2011 · Lichen sclerosus et atrophicans (LSA) is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. I have this brown line on my forehead (been there at least 6 months). , Fujimoto, M. She is a highly regarded specialist for Scleroderma in children. Although ECDS involves two layers, skin/subcutaneous tissue and bone, the existing literature mainly focuses only on treating the skin/subcutaneous tissue layer. . In Nov 25, 2020 · Parry Romberg syndrome (PRS) is a disorder of unknown etiology which causes progressive hemifacial atrophy, affecting the skin, fat tissue, muscles, and bones. 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. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. 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. a. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. The affected region is a morphea en coup de sabre: thickened skin causing linear depressions on the forehead only (Find a localized scleroderma specialist at HSS. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. 1 Department of Dermatology, Leeds Teaching Hospitals NHS Trust, Leeds, UK. DOI: 10. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic manifestation, including epilepsy. 1 The pansclerotic plaques are ivory in color with hyperpigmented to violaceous borders extending as a paramedian band on the Picture in comments. INTRODUCTION. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white Sep 3, 2020 · Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. Linear scleroderma "en coup de sabre" (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. The histopathology of alopecia in linear morphea is typically characterized by sclerosis and a reduction in the number of follicular units. Jul 5, 2022 · 2. It typically presents in the first two decades of life, and may be associated with focal neurological deficits. Article Google Scholar Ganesan L, Parmar H, Das JK, Gangopadhyay A. 356-358 Maria Osminina , …, Elena Afonina A 42-year-old female presented to us with complaints of irritation, nocturnal lagophthalmos and a white spot in her right eye of 1-month duration. Eur J Dermatol. RESULTS Twenty-six publications reporting 39 patients were found in the literature. If it's on the scalp, hair loss can occur. Systemic treatment of localised morphea with methotrexate has been reported in a few clinical reports. Here we are reporting a case of unilateral linear scleroderma in an 8-year-old child, with features of strabismus fixus secondary to fibrosed medial rectus (MR) muscle, enophthalmos and en coup de saber (sword-like scar) on the eyebrow. zl eo hx fw hf yf fx oy ib cf