Scleroderma en coup de sabre

Scleroderma en coup de sabre. Linear morphea occurs in a line usually on the head, arm or leg. " "Linear scleroderma is one sub-type of localized scleroderma, most commonly occurring in childhood. Involvement of internal organs or the transition to systemic sclerosis is Mar 13, 2019 · Extracutaneous findings in 22% to 56% (Figure 10): Arthritis, myalgias, neuropathies are most common; neurologic and ocular complications (potential for seizures and vision changes/loss) in linear morphea with en coup de sabre; dental malocclusion tongue and salivary gland atrophy in lesions involving the face. Figure 10. Nov 4, 2019 · To provide an update on the current management, including evaluation and treatment, and the available diagnostic tools for linear scleroderma of the head, i. ) Systemic scleroderma (systemic sclerosis) Dec 1, 2009 · Linear scleroderma is a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed prior to 18 years of age. Rai R et al (2000) Bilateral en coup de sabre-a rare entity. 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. Iran J Child Neurol. While the pathophysiology of cutaneous linear scleroderma includes endothelial cell injury and up regulation of pro-fibrogenic pathways, the Jul 22, 2014 · The scleroderma en coup de sabre is a variant of linear or localized scleroderma that occurs preferentially in children. En coup de sabre is an unusual variant of localized scleroderma characterized by its distinct location involving the frontoparietal region of the forehead and scalp. Linear Scleroderma “en coup de sabre”. , Parry-Romberg Syndrome and en coup de sabre (PRS/ECDS). We describe a patient with scleroderma en coup de sabre in whom intracerebral and orbital lesions developed after 2 decades of disease duration. Often, the skin lesion is not quite hard to Dec 4, 2001 · Background 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. 0544. In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. The patients with deeper lesions can develop ocular and neurologic symptoms like headaches and seizures and very rarely develop progressive hemifacial atrophy. Children with linear scleroderma will often have a positive outlook, provided that they receive appropriate and prompt treatment to minimise any longer-term complications. 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). Mertens JS et al. 1 , 2 Its etiology is unknown, but probably it is of autoimmune nature. En coup de sabre scleroderma and Parry-Romberg syndrome in adolescents: surgical options and patient-related outcomes. A case of scleroderma 'en coup de sabre' presenting with uveitis, predominantly posterior, and intractable grand mal seizures is described. Introduction Linear scleroderma “en coup de sabre” (ECDS) is a rare subset of localized scleroderma. When it crosses a joint there may be resultant immobility. 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. En coup de sabre (ECDS) is a form of linear-scleroderma, primarily affecting the face. Some authors consider these to be two different diseases. The "en coup de sabre" subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Central nervous system (CNS) involvement has been increasingly recognized in these disorders, denoting a vast spectrum of neurological manifestations, such as seizures Sep 27, 2018 · The study, “ Enhancement of the aesthetic outcome of scleroderma en coup de sabre with botulinum toxin injection,” was published in the Journal of the American Academy of Dermatology Case Reports. En coup de sabre (linear atrophic band +/- sclerosis, classically on the forehead or scalp) Parry Romberg / progressive hemifacial atrophy (atrophy of the fat, muscle and bone +/- dyspigmented +/- sclerotic changes, affecting one side of the face). A rapid scoping review of the literature was conducted to include manuscripts published in English between 2010 and 2019. Current treatment recommendations include topical, intralesional, and oral Nov 27, 2021 · C) linear scleroderma and D) en coup de sabre. This article presents the clinical manifestations of Jul 29, 2011 · Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. Background: To evaluate ophthalmic involvement in a long-term series of patients with en coup de sabre (ECS) close to the eye based on the hypothesis that this is not commonly affected, or simply under-reported. May 11, 2004 · Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. A subtype of LoS including lesions in the head is further classified as linear scleroderma en coup de sabre (LSeCS). It may involve all of the hemiface, extending from the scalp to the the nose, lips and tongue. 3–4 ), or can be limited to the forehead or scalp, where is it known as en coup de sabre ( Fig. Nov 25, 2020 · Facial and oral changes in Parry Romberg Disease with En Coup de Sabre Scleroderma: En coup de sabre lesion of the forehead, left facial soft tissue atrophy, atrophied left nasal wing and lip atrophy: (a) front view at age 4 and (b) front view at age 10. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution on the frontoparietal scalp with surrounding scarring alopecia. In both cases, the slides were randomly presented and no clinical information was given. Bernard-Medina b, F. Various ophthalmological and neurological abnormalities have been observed in patients with LSCS. Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Linear scleroderma “en coup de sabre” (LSCS) is a subset of linear scleroderma, affecting frontopariental region, with frequent neurological and ophthalmological involvement. Jul 29, 2011 · Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. Clinically, she had epilepsy, impaired vision, and retro-ocular pain of the affected eye. It is usually treated with methotrexate and corticotherapy in addition to folic acid supplements. Background: En coup de sabre (ECDS) is a form of linear-scleroderma, primarily affecting the face. Oct 12, 2023 · PRS is considered a subset of linear morphea, a type of localized scleroderma. doi: 10. Localized scleroderma is also referred to as Morphea, and is differentiated from systemic sclerosis by the absence of sclerodactyly, Raynaud’s phenomenon, capillaroscopic abnormalities, and Abstract. All patients had craniofacial scleroderma: linear scleroderma en coup de sabre (LScs), progressive facial hemiatrophy (PFH, or Parry-Romberg syndrome) or both. It can occur as a single lesion, usually on an extremity, can be widespread (Fig. 1 When linear scleroderma occurs on the head, it is referred to as linear scleroderma “en coup de sabre” (LSCS). A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic Feb 1, 2017 · Abstract. According to the literature, involvement of the ‘on line’ eye is rare, often just minor, and noticed mainly as an occasional finding. Affected individuals typically have a characteristic atrophic skin lesion involving the fronto-parietal scalp. retrospective analysis of 344 patients with paediatric- or adult-onset disease. Central nervous system involvement is not rare and neurological manifestations include seizures, focal neurological deficits, headache and neuropsychiatric changes. Summer 2015;9(3):62-68. May 20, 2021 · Background: Localized scleroderma en coup de sabre (LScs) is a form of localized scleroderma thought to be an autoimmune disorder. The affected region is a depression Jul 1, 2016 · Localized scleroderma en coup de sabre (LScs) is a form of localized scleroderma, involving the fronto-parietal area, with band-like sclerotic lesions with atrophy, furrow of the skin and outer diploe thinning ( Unterberger et al. If it's on the scalp, hair loss can occur. 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. The lesions may follow lines of Blaschko. May 3, 2019 · A 35-year-old Dinka woman presented with left hemifacial atrophy associated with left-sided body hemihypoesthesia and glaucoma with overlapping linear scleroderma “en coup de sabre” for 5 years. When it occurs on the scalp or forehead, it is termed "en coup de sabre". Oct 20, 2022 · Scleroderma en coup de sabre URL of Article 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. 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. A patient wit … 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. González-Cornejo a LINEAR SCLERODERMA EN COUP DE SABRE. Methods: An observational study of ophthalmological findings in patients from Copenhagen University Dermatology Clinics. A total of 34 articles analyzed treatment outcomes for patients with localized scleroderma en coup de sabre including 4 retrospective cohort studies, 2 prospective cohort studies Jul 4, 2018 · Scleroderma en coup de sabre typically presents with a cutaneous induration, but involvement of the underlying bone, eye, and brain is common. Jan 27, 2012 · Linear scleroderma en coup de sabre (LCsc) is a rare subset of LS. Objective: Review the existing surgical tools for the correction of "en coup de sabre" ECDS lesions and facial Jul 4, 2018 · Linear scleroderma of the face (en coup de sabre) is a disfiguring chronic skin disorder, often starting in childhood or adolescence. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and Nov 3, 2008 · Literature search yielded 54 case descriptions of localized scleroderma associated with neurologic symptoms and neuroimaging findings. Localized scleroderma (LoS) is a rare condition featuring skin and underlying tissue sclerosis not usually compromising other systems. Follow-up visit after 2 weeks showed a reduction in epithelial bullae. ijd_992_22. This case describes a 36-year-old female who presented with a history of en coup de sabre with 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. [3][4] [5] As with our patient, these are typically ipsilateral to the cutaneous findings. Many patients have comorbid central nervou …. Resistance to conventional immunosuppressive therapy forced us to initiate the treatment with tocilizumab. Organic intracranial abnormalities in this disease are well-documented May 19, 2020 · Linear scleroderma “en coups de sabre” is a disease that causes scar-like lesions in the forehead and the scalp, and atrophy of the underlying structures. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Lesions form depressed grooves that resemble a sword wound or the stroke of a saber. Br J Dermatol 172:722-728. 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. The exact cause is still unknown. OBJECTIVE . Despite effectiveness of pharmacological interventions in the management of lesions, sequelae of cutaneous deformities are common. Scleroderma en coup de sabre can result in neurological symptoms such as headache or seizures and may even cause structural alterations in the brain. NOTA CLÍNICA Esclerodermia lineal en coup de sabre. 5… To differentiate between scleroderma 'en coup de sabre' (SCS) and progressive facial hemiatrophy (PFH), colour slides of 13 patients diagnosed as SCS and nine as PFH were examined by two dermatologists and microscopic slides by two pathologists. Review the existing surgical tools for the correction of “en coup de sabre” ECDS lesions and facial deformities. 4103/ijd. We report on 4 pediatric cases with atypical initial On the diagnosis of scleroderma en coup de sabre, prednisolone intake was initiated, and the skin lesions were well controlled. 1 ECDS typically occurs in children and is characterized by a 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. In most cases, Raynaud's phenomenon is absent. Rare complications of scleroderma en coup de sabre are orbital and intracerebral involvement. 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. If there is involvement of the head, the linear scleroderma is referred to as en coup de sabre (LScs) . , 2008). All manifestations are primarily due to chronic progressive fibrosis of the skin or structures close to the skin. Scleroderma, Localized / therapy. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. Aims Mar 21, 2024 · En coup de sabre is a rare form of linear scleroderma that affects the head, especially the forehead. The problem of differential diagnosis of PHA and ECDS is being discussed ( 12 , 13 ). En coup de sabre (ECDS) is a rare form of localized scleroderma that typically manifests in children and women. Scleroderma en Coup de Sabre Dtsch Arztebl Int. 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. Aims Background: 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. Earlier it was highlighted that PHA and linear scleroderma “en coup de sabre” (ECDS) often coexisted (6, 8), but some authors described PHA as a unique disease (9–11). LScs and PFH should be viewed as variants of Sep 29, 2021 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Linear alopecia areata may present as a close Jan 17, 2012 · “En coup de sabre” is an extremely rare linear form of localized scleroderma that was first described in 1854 by Addison and has an incidence of 0. Background:LScs is a rare subset of localized scleroderma. Recent reports confirm that central nervous system involvement is not rare, and presentations include Sep 29, 2021 · The keywords en coup de sabre, “facial linear scleroderma”, and “morphea linearis”, combined with “treatment” or “therapy” were used as search terms. Hautarzt 45: 398-401. Romero-Vargas a, G. Introduction. Ruiz-Sandoval a, S. Sep 3, 2020 · Linear scleroderma usually manifests as cutaneous linear scar-like lesions of the head or limbs . The case of a 54-year-old woman with a 3 and one-half year history of en coup de sabre is presented. There is controversy about whether en coup de sabre is truly a variant of linear 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. The result is an acute facial asymmetry that can be distressing to affected young adults. The disease may be associated with facial hemiatrophy. However, in the course of our follow up, he had another episode of brain hemorrhage, again without any evidence of cerebral vascular abnormalities. A. When the face is affected, some strips located on the forehead may be hollow and lead to an appearance termed ""en coup de sabre"". 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 Previously considered a rare event, nervous system involvement in scleroderma has been increasingly recognized. The disease usually has a benign course A case of scleroderma 'en coup de sabre' presenting with uveitis, predominantly posterior, and intractable grand mal seizures is described. We report on 4 pediatric cases with atypical initial Linear scleroderma en coup de sabre (LSCS) is a clinical variant of morphea that presents with a linear alopecic patch over the frontal scalp. … as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. Sep 29, 2021 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Neurologic involvement is common and should prompt a comprehensive neurologic workup in patients suspected to have ECDS or PHA. En coup de sabre is a linear scleroderma that presents on the frontal or frontoparietal scalp. (2015) Disease recurrence in localized scleroderma: a. It is the most frequent form of scleroderma encountered in children . 2023 May-Jun;68(3):346-348. The clinical presentation, laboratory findings, epidemiology, etiologic hypotheses, and treatment options are reviewed. Ostertag JU et al. 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. The databases May 2, 2007 · Each of these subtypes can involve the face and particularly the orbit. Linear scleroderma may coexist with progressive facial Mar 8, 2023 · Ostertag JU et al (1994) Bilateral linear tempoparietal scleroderma en coup de sabre. She was advised topical 1% prednisolone acetate eye drop in a tapering dosage along with night time patching. Case history of a small girl outlet with epilepsy, followed by scleroderma skin damage and uveitis, neurovasculitis with white matter foci in brain on the side of skin lesion in two months, immunologic disease activity. Jul 27, 2009 · The connections of Parry-Romberg syndrome with scleroderma linearis or with en coup de sabre – typed limited scleroderma are often discussed. Linear scleroderma tends to involve the underlying fat and muscles and may lead to muscle atrophy. García-Navarro a, A. Mar 19, 2021 · It can be subdivided into linear, circumscribed (plaque), generalized, pansclerotic, and mixed subtypes. 13 cases per 100,000 population based on the landmark Mayo Clinic epidemiological study over a 33-year follow-up . Palmero ML, Uziel Y, Laxer RM, Forrest CR, Pope E J Rheumatol 2010 Oct;37(10):2174-9. ” Approximately one-third of people with linear scleroderma on their head or neck also have Parry-Romberg syndrome. Abstract. linear scleroderma: lines or bands of thickened skin on the face, arms or legs; morphea en coup de sabre: thickened skin causing linear depressions on the forehead only (Find a localized scleroderma specialist at HSS. Parry–Romberg syndrome is a very rare entity causing progressive hemifacial atrophy that could also be associated with linear scleroderma. L. 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. Dec 4, 2001 · BackgroundLinear 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. Linear scleroderma en coup de sabre; This arises when children have linear scleroderma presenting on their face and sometimes their scalp. Cerda-Camacho c, R. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. 1 , 3 , 4 Besides the skin lesions, neurologic and Linear scleroderma “en coup de sabre” (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. Neurological involvement in LSeCS can reach up to 4% and may include seizures. Feb 16, 2024 · Localized scleroderma (LS), also called circumscribed scleroderma or morphea, comprises a heterogeneous group of diseases that can be classified into four subtypes: limited, linear, generalized, and mixed LS. For some however, hemifacial atrophy is an intensified form of linear scleroderma [1,5]. We describe a 15-year-old g … . G. Morphea is distinct from systemic sclerosis (scleroderma),… percent; Generalized ‒ 8 percent; Linear ‒ 6 percent; En coup de sabre (a form of linear disease) ‒ 3. Imaging findings and histopathology indicated that the process, most likely focal inflammatory, may be progr … Localized scleroderma is a rare disease, characterized by sclerotic lesions. Methods: Patients attending the Neurology Clinic 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. Dermatologist 45: 398-401; Polcari I et al (2014) Headaches as a presenting symptom of linear morphea en coup de sabre. We describe an unusual case of LSC. 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. A diagnosis of linear scleroderma en coup de sabre (LSCS) with focal corneal edema was made. Morphea is classified into circumscribed, generalized, linear, and pansclerotic subtypes according to the clinical presentation and depth of tissue involvement. A variety of presentations have been described, with different clinical characteristics and specific prognosis. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup de sabre. 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 one or a few (3-4) patches of skin thickening with different degrees of pigment changes. Jan 1, 2020 · Linear scleroderma en coup de sabre (LSCS) is a form of localized scleroderma associated with band-like fibrotic lesions involving the frontoparietal area (Kister et al. Epilepsy, when present, is related to these brain lesions. [3] 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 . Jan 1, 2021 · Scleroderma en coup de sabre typically presents with a cutaneous induration, but involvement of the underlying bone, eye, and brain is common. This form of scleroderma is infrequent, mainly in pediatric patients, but not infrequently associates neurologic symptoms, especially epilepsy. Case presentation A 23 year old woman Mar 8, 2023 · Eur J Dermatol 13: 171-176. , 2003 ). Dec 19, 2022 · The “en coup de sabre” is French for “cut from a sword. Cerebral changes both in scleroderma en coup de sabre and PFH are mainly related to vascular involvement (neurovasculitis) [2, 13]. Wide strips mainly develop on the face, thorax, arms, and legs. Seizures and headache are the most reported features in LS en coup de sabre, while peripheral and autonomic nervous systems involvement predominate in SSc. 2019. This is the most frequent form of morphea of the scalp. (1994) Bilaterale lineare tempoparietale Sklerodermie en coup de sabre. Mar 10, 2024 · En coup de sabre (ECDS) is a rare variant of linear scleroderma typically found among children. Here, we … Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. Pediatr Dermatol 17: 222-224. Mar 27, 2010 · En coup de sabre is a linear scleroderma that presents on the frontal or frontoparietal scalp. May 19, 2020 · Linear scleroderma “en coups de sabre” is a disease that causes scar-like lesions in the forehead and the scalp, and atrophy of the underlying structures. The authors describe a rare case of en coup de sabre in a 3-year-old boy whose disease onset was at 12 months of age. 3238/arztebl. In this paper, clinical presentation of LScs and its neurological involvement are described. Pediatrics 134: e1715-1719 . A Case of Linear Scleroderma En Coup de Sabre Accompanied with Migraine with Visual Aura and Idiopathic Intracranial Hypertension Indian J Dermatol . A) Morphea – This is the most common type of localized scleroderma. Once thought to be an Nov 1, 2005 · En coup de sabre or linear scleroderma has been associated with imaging findings in several reports. Manifestaciones neurológicas, imágenes y revisión J. Linear scleroderma presents as linear indurated plaques with dyspigmentation on the limbs, head (en coup de sabre), or rarely the trunk. 2019 Aug 5;116(31-32):544. This case describes a 36-year-old female who presented with a history of en coup de sabre with Jun 17, 2020 · Morphea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissues, or both. Linear scleroderma en coup de sabre (LSCS) is a lesion in the frontoparietal scalp that also falls on the spectrum of localized scleroderma and is often mistaken for PRS due to their similar clinical appearance. e. 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 differential diagnosis includes, for example, hemiatrophia faciei progressiva. 3–5 ). In all of these children the disease started at the very early age, in most of them after trauma, believed to En coup de sabre is a type of linear scleroderma that presents on the frontal or frontoparietal scalp. Aug 5, 2019 · Scleroderma en Coup de Sabre. Linear scleroderma is most common in young girls. Some areas are dark while others are lighter than the surrounding normal skin. The typical presentation affects frontoparietal region, and the mean age of onset is around 13 years old . Gutiérrez-Aceves a, V. In our patient, gliosis in the area of the Scleroderma en coup de sabre is a descriptive term denoting linear scleroderma of the frontoparietal area of the face and scalp. 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. Several surgical treatments are available such as free tissue transfer and synthetic fillers. In most cases, it occurs as a single paramedian line that may be associated with hypoplasia of underlying structures and hemiatrophy of the face. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. In our patient, gliosis in the area of the basal ganglia was diagnosed with the aid of magnetic resonance imaging. In the case of facial involvement, some strips developing on the forehead may be hollow and lead to an appearance termed en coup de sabre. We present 4 children with double lines of scleroderma en coup de sabre at the frontal area (Figure 1). Riestra-Castañeda a, S. Authors Robin Reschke Linear scleroderma “en coup de sabre” is a rare subset of localized scleroderma, a distinct and separate disease entity from systemic scleroderma . Mar 9, 2020 · Linear morphea/linear scleroderma . Analysis of CSF and pathology obtained at Linear scleroderma generally first appears in young children. es bz hx jj hf vv xz yl fa gz