1 It is more common in females and the pathogenesis is not completely understood. Introduction Linear scleroderma “en coup de sabre” (ECDS) is a rare subset of localized scleroderma. It can be associated with hemiatrophy of the tongue. Paramedian lesions are more common than median lesions. Hence Dec 12, 2006 · Institutional review board approval was obtained for this study. Aug 25, 2022 · Morphea (mor-FEE-uh) is a rare condition that causes painless, discolored patches on your skin. The keywords “en coup de sabre”, “facial linear scleroderma”, and “morphea linearis” Jan 19, 2024 · Als Sklerodermie „en coup de sabre“ (Säbelhieb-Form) wird die Manifestation im Bereich des Gesichts und des behaarten Kopfes bezeichnet (Abb. Here we describe 4 patients with ECDS who presented with headaches, which were typical migraines in 3 of the patients morphea: roundish or irregular-shaped hard patches of skin on the face, hands, feet or trunk; 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. This form of localized scleroderma manifests through an inflammatory phenomenon of collagen overproduction and extracellular matrix destruction, affecting the skin and subcutaneous tissues of the face. This type is often found in the arms, legs, and forehead (frontoparietal: “coup de sabre”) or in many other areas. 1, 11 The lesions commence as of localized scleroderma en coup de sabre. Sep 29, 2021 · Reconstructive and surgery treatment was successfully used for lesions with settled disease activity to improve the cosmetic aspect of the lesions and methotrexate was the most commonly investigated treatment. It presents as one or a few (3-4) patches of skin thickening with different degrees of pigment changes. Sep 30, 2016 • 9:35 AM. The aim of this article was to systematically analyze cu … Aug 1, 2022 · 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. Ocular involvement in children with localised scleroderma: a multi-centre study. 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). 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 Sep 29, 2021 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Jun 1, 2018 · Abstract. … as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. Four (7. In 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. Mar 19, 2021 · The en coup de sabre variant of linear scleroderma typically occurs in children. Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women. 4317/jced. Many patients have comorbid central nervou …. Systemic treatment of localised morphea with methotrexate has been reported in a few clinical reports. The histopathology of alopecia in linear morphea is typically characterized by sclerosis and a reduction in the number of follicular units. Our study is the first to describe the surgical options and the outcomes of surgical interventions in patients with facial morphea. 10 While single lesions are most common, multiple lesions may coexist in a single patient, with reports suggesting that the lesions may follow Blaschko's lines. Current treatment paradigms rely on low levels of evidence, primarily case Abstract. Jul 28, 2021 · Individuals with the severe inflammatory subtypes, en coup de sabre linear scleroderma, or Parry-Romberg disease can have severe functional limitations. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup de sabre. 1%) had Parry-Romberg syndrome, and 15 (27. Kennzeichnend ist eine Verhärtung der Haut, die sich säbelhiebförmig von der Kopfhaut über die Stirn ziehen und bis hin zur Augenbraue reichen kann. 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. In literature, en coup de sabre morphea, and en coup de sabre scleroderma are terms used interchangeably and synonymously. This may cause severe contractures that result in limited movement and permanent disability of an arm or leg. Before and After 2 Sessions (1,910 Grafts) Jun 14, 2023 · En coup de sabre is a rare subtype of morphea. However, linear morphea, including the en coup de sabre subtype, may remit and reactivate, remain unchanged, or become more extensive with time. Loss of eyebrow or eyelashes, ptosis, pseudooculomotor palsy, uveitis, asymmetry of the tongue, and altered dentition and dental caries can occur in patients with morphea en coup de sabre. Aug 1, 2022 · The term en coup de sabre morphea refers to a lesion of linear morphea typically located in the. Affected individuals typically have a characteristic atrophic skin lesion involving the fronto-parietal scalp. Beperkt tot één helft van het gelaat. 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. Figure 10. Damage to underlying brain and eye tissue is a potential, serious complication of the en coup de sabre subtype. En coup de sabre is a rare form of linear scleroderma that affects the head, especially the Linear morphea en coup de sabre is a localized form of morphea that presents as paramedian face or frontal scalp depression. Pediatrics 134: e1715-1719 . The Then, plaques (morphea) or wide strips (linear scleroderma) appear. The head variant termed “en coup de sabre” (LSES) is characterized by an indurated long streak resembling the deep wound of a sword. Pediatr Dermatol 17: 222-224. We report 2 adult women with LSCS with distinct dermoscopic findings. However, in about 32% of cases, it may arise in Sclerodermie 'en coup de sabre' is een lineaire sclerodermie van het gelaat. Frontoparietal scleroderma (en coup de sabre) following Blaschko’s lines. Jun 6, 2011 · Morphea, also known as “localized scleroderma” (a term that should be discouraged because of unnecessary confusion with scleroderma among providers and patients), is an inflammatory condition that primarily affects the dermis and sometimes extends into subcutaneous (SQ) fat and fascia producing thickening and hardening of the skin. En coup de sabre can also be disfiguring. , Witman, P. 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. In children, linear morphea on the arms and legs can affect the underlying bone and interfere with bone growth. Heeft alle kenmerken van lokale sclerodermie, met depressie van de huid ter plaatse. The true incidence of this disorder has not been reported, but it is often regarded as a subtype of localized scleroderma. Linear scleroderma may coexist with progressive facial 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. Sklerodermie en coup de sabre Die Sklerodermie en coup de sabre ("Säbelhieb-Sklerodermie") ist eine Sonderform und tritt meist im Kindesalter auf. Only a few bilateral cases have been reported to date. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution on the frontoparietal scalp with surrounding scarring alopecia. En coup de sabre (ECDS) and Parry-Romberg syndrome (PRS) are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. Analysis of CSF and pathology obtained at In en coup de sabre type, the patient may have neurological abnormalities like migraine, trigeminal neuralgia, seizures, behavioral changes, and learning disability. It may occur on one side of the body only. extremitätenbetonten Formen der linearen LoS oder bei Kopf-Hals-Beteiligung wie bei „en coup de sabre“, Parry-Romberg-Syndrom, aber auch anderen, schwergradig verlaufenden Formen mit einer muskuloskeletalen Beteiligung sollte eine MRT(Magnetresonanztomographie)-Untersuchung veranlasst werden. 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. 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. The lesional skin first exhibits contraction and stiffness that lead to characteristic fibrotic plaques with associated linear alopecia. Morphea is clinically divided into linear, circumscribed (plaque), generalized, pansclerotic, and mixed forms. The existence of neurologic findings in association with ECDS has been well described in the literature. Wide strips mainly develop on the face, thorax, arms, and legs. 8% (15/109) of those with head and neck involvement had features of both en coup de sabre and Parry-Romberg syndrome. Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions 7 Soma, Y. Morphea is distinct from systemic sclerosis ( scleroderma ),… percent; Generalized ‒ 8 percent; Linear ‒ 6 percent; En coup de Mar 18, 2013 · En coup de sabre form of morphea often affects the scalp with thickening, sclerosis, dyspigmentation, and scarring alopecia. Linear morphea on the head (also called en coup de sabre) may extend inwards to the brain and cause seizures. In rare cases, the larger lesions can merge together, covering the entire body. 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. Demographic data, examination findings, and Mar 21, 2024 · Bullous morphea causes blister-like bumps or areas of erosion on the region of morphea. The groove may extend from the scalp and forehead down to the cheek, nose, upper lip, and rarely the chin, neck, jaw, and tongue. Oct 16, 2022 · Morphea en coup de sabre is a rare pathology that causes significant aesthetic damage to the face and for which, to date, no curative treatment has been found yet. 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 Jan 27, 2016 · Zu den Subtypen mit extrakutanem Befall gehören die lineare ZS mit den Unterformen lineare ZS von Typ en coup de sabre und progressive faziale Hemiatrophie, die generalisierten Formen mit den Unterformen disabling pansclerotic morphea und eosinophile Fasziitis, sowie die tiefe Form (deep morphea). , Fujimoto, M. 1b, c). Of note, 13. 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 Dec 15, 2012 · Linear morphea en coup de sabre (ECDS) is a form of localized scleroderma that predominantly affects the pediatric population, with a median age of 10 years at presentation. Objectives: To describe the neuroimaging and clinical findings in patients with localized scleroderma en coup de sabre (LScs). The databases Abstract. I was wondering if any one else out there has this. Oct 21, 2021 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. The patient was treated with oral steroids and oral methotrexate improving her cutaneous disease. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Zelfs de tong en het gebit kan meedoen. We report a unique case of adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda. 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. ings consistent with en coup de sabre morphea, 13 (24. There is a female to male predominance in morphea of approximately 3 to 1 [ 1 ]. En Coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. ) Systemic scleroderma (systemic Feb 22, 2022 · Dear Editor, MORPHEA EN COUP DE SABRE AND PARRY–ROMBERG SYNDROME: WHEN IT IS WRITTEN ON THE FACE. 2. There is controversy about whether en coup de sabre is truly a variant of linear Mar 6, 2018 · Abstract. Odom1. A 24 year old woman in the 33rd week of pregnancy developed progressive neurological complications with right sided hemiparesis in association with the occurrence of linear scleroderma “en coup de sabre” (LSCS) and pre-existing plaque-morphea, already being treated by balneophototherapy. Scleroderma, Localized / therapy. This active pathological process is challenging to control and often goes unnoticed at its early stage. Oct 1, 2010 · En coup de sabre scleroderma and Parry-Romberg syndrome are 2 relatively rare forms of facial morphea that result in loss of facial contour and symmetry, requiring surgical intervention. frontoparietal scalp and/or the paramedian forehead, often resembling a strike with a sword. Jan 10, 2022 · Both the en coup de sabre (ECDS) and progressive hemifacial atrophy (PHA) variants of linear morphea have median onset at age of 10 and 13. This active pathological … Feb 16, 2024 · Gerade bei den gelenkübergreifenden bzw. Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults. Oct 29, 2013 · The en coup de sabre variant of linear morphea presents as an atrophic linear streak that is most often located on the paramedian forehead and scalp. May 31, 2019 · En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. Methods: Patients with LScs were evaluated by high-resolution MRI and CT. 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 The balance between the technical aspect of placing the new grafts with a certain depth and density into this rather compromised area and, of course, the emphasis on the final aesthetic successful outcome, which created a natural-looking hairline, especially for a young maturing female. J Am Acad Dermatol 2007; 56:257. J Am Acad Dermatol 1998;24:467-8. 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. Apr 17, 2019 · Of those with linear lesions located on the head or neck (N = 109), 65. 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. Jul 6, 2023 · En coup de sabre (ECDS) is a form of linear scleroderma or morphea that distinctly appears on the forehead and/or frontoparietal scalp. While the pathophysiology of cutaneous linear scleroderma includes endothelial cell injury and up regulation of pro-fibrogenic pathways, the Linear scleroderma. Here we describe 4 patients with ECDS who presented with headaches Dec 1, 2014 · Linear morphea en coup de sabre (ECDS) is a form of localized scleroderma that predominantly affects the pediatric population, with a median age of 10 years at presentation. The lesion is usually unilateral and mostly affects the Jul 4, 2018 · What was known before. A 4-year-old girl was referred to the dermatology department with a 3-year history of a linear patch of alopecia and a sunken-in appearance of the left side of the face. Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. Plaques are round or oval, often with a white appearance and a red rim. 1 PRS usually presents in the first decade of life, but later presentations have been described. A dermatologist can use a filler, such as hyaluronic acid, to restore the face. The disease usually has a benign course Jul 1, 2019 · PDF | On Jul 1, 2019, Tasleem Arif and others published Morphea «En Coup De Sabre» at the Site of Healed Herpes Zoster Ophthalmicus | Find, read and cite all the research you need on ResearchGate Oct 16, 2022 · Linear scleroderma en coup de sabre is a rare chronic autoimmune disease. A) Morphea – This is the most common type of localized scleroderma. Dermatologist 45: 398-401; Polcari I et al (2014) Headaches as a presenting symptom of linear morphea en coup de sabre. Jun 17, 2020 · Frontoparietal linear morphea, called en coup de sabre, is characterized by a linear, atrophic depression affecting the frontoparietal aspect of the face and scalp, suggestive of a stroke from a sword, as shown in the image below. Linear morphea en coupe de sabre (LM) usually arises in childhood. ” Depending on where this groove is, it can cause serious issues, such as seizures and problems with the teeth, jaw, nose, lips, and eyes. Mar 1, 2007 · 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 tends to have a waxing and waning course. Rai R et al (2000) Bilateral en coup de sabre-a rare entity. 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. ECDS has a specific distribution on the frontal scalp and forehead and is usually unilateral. 6 years, respectively . Typically, the skin changes appear on the belly, chest or back. May 10, 2004 · Abstract. 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. Meestal in fronto-parietale gebied, kan ook uitbreiden naar wang, neus, lip en kin. 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. Nov 27, 2021 · types are: A) morphea B) generalized morphea C) linear scleroderma and D) en coup de sabre. It manifests by presence of linear atrophy and/or hardening of the skin, subcutis, occasionally involving muscles and bones [ 2 ]. Sep 30, 2016 · Morphea en coup de sabre. However, through our study, autologous transfer of adipose tissue appears to be a promising therapeutic approach for both morphological and functional improvements to this pathology. 8%) patients had both. It took over a year to diagnose and have many lesions on my head. Zannin ME, Martini G, Athreya BH, et al. Summer 2015;9(3):62-68. 1 Neurologic symptoms are the most Nov 9, 2016 · However, linear-type localised scleroderma, especially the en coup de sabre subtype, may become more extensive with time. Have tried light therapy,steroids but nothing seems to slow it down. Morphea tends to affect only the outer layers of your skin. The face is the most common site for the linear type of morphea with fibrosed tissue appearing as a sabre cut (en coup de sabre) resulting in facial hemiatrophy mimicking Parry–Romberg syndrome . Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Mar 18, 2013 · Linear morphea en coup de sabre is a localized form of morphea that presents as paramedian face or frontal scalp depression. 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. But they might also appear on your face, arms and legs. Methods: An observational study of ophthalmological findings in patients from Copenhagen University Dermatology Clinics. En coup de sabre (ECDS) is a rare form of localized scleroderma that typically manifests in children and women. According to the literature, involvement of the ‘on line’ eye is rare, often just minor, and noticed mainly as an occasional finding. However, there are no case series using methotrexate treatment for En coup de sabre. Oct 16, 2022 · Linear scleroderma en coup de sabre is a rare chronic autoimmune disease. Any suggestions of what others have tried I would love to hear. Oct 24, 2016 · Mixed forms, such as localized scleroderma of the face with plaque/linear morphea at some other site, have been reported in the literature . Results: Nine Dec 1, 2009 · Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. I have morphea en coup de sabre. May 16, 2019 · LS encompass 4 main categories: circumscribed morphea, generalized morphea, pansclerotic morphea and linear morphea, which is further subdivided into trunk/limb and head variants. Linear scleroderma of the face (en coup de sabre) is a disfiguring chronic skin disorder, often starting in childhood or adolescence. Feb 1, 2017 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. The ocular abnormalities include absence of eyebrows and eyelashes on the affected areas, enophthalmos, anterior uveitis, and myopathy of the eye muscles. J). Generalized morphea may also limit joint function due to its larger coverage areas. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. Feb 13, 2023 · Linear morphea of the head/face and neck area causes an atrophic depression of the skin, often involving also the underlying soft tissue, bone and brain, characteristically resembling the cut from a sword, therefore also known as linear morphea en coup de sabre (Figures 2I, ,J). Mar 19, 2021 · Keywords: Scleroderma, en coup de sabre scleroderma, adult-onset scleroderma, linear scleroderma, morphea, localized scleroderma Introduction Localized scleroderma, also known as morphea, is a cutaneous disorder characterized by fibrosis of the skin and less frequently of the underlying tissue. They both had an atrophic well-defined linear patch of alopecia on the frontotemporal scalp. Niklander and Constanza Marin and Ren{\'e} Martinez and Alfredo Esguep}, journal={Journal of Clinical and Experimental Dentistry}, year={2017}, volume={9}, pages={e315 Feb 12, 2019 · PRS and morphea en coup de sabre (ECDS) have significant overlap, often coexist, and are likely different phenotypes of morphea. 2% (34/109) had Parry-Romberg syndrome, also known as hemifacial atrophy. Linear scleroderma “en coup de sabre” (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. If morphea crosses a joint, the thickening may limit joint movement. Hierbei kommt es zu einer strangförmigen Atrophie, lokaler Hyperpigmentierung, Hautverdickung und Haarverlust des Gesichts, der Augenbrauen, der Stirn und der behaarten Kopfhaut; der darunterliegende Knochen ist typischerweise mit betroffen. Jun 9, 2023 · It has a significant impact on the patient's daily life due to the unaesthetic evolution of the cutaneous lesions. A person with a mild case develops a bit of tissue loss. 53151 Corpus ID: 14458926; Morphea “en coup de sabre”: An unusual oral presentation @article{Niklander2017MorpheaC, title={Morphea “en coup de sabre”: An unusual oral presentation}, author={Sven E. Linear Scleroderma “en coup de sabre”. En coup de sabre: A line of thickened skin forms on the scalp, face, or both, and the tissue beneath disappears. En coup de sabre (ECDS) is a rare subtype of linear scleroderma that is limited to the hemiface in a unilateral distribution. Methods: A total of 16 patients with ECDS were retrospectively evaluated on the basis of clinical and histological findings. Methods Scopus, PubMed, and EBSCO databases were searched for all reports discussing the treatment of localized scleroderma en coup de sabre published up to 4 January 2021. 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. . Mar 8, 2023 · Ostertag JU et al (1994) Bilateral linear tempoparietal scleroderma en coup de sabre. Nov 14, 2023 · This is called “linear morphea en coup de sabre,” French for “the blow of a sword. The sequelae DOI: 10. Traditionally, it has been thought that the alopecia is not responsive Linear lesions tend to persist for longer than plaque-type lesions, but they often improve over the years. It manifests by presence of linear atrophy and/or hardening of the skin, subcutis, occasionally involving muscles and bones. Sep 29, 2021 · Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women . Morphea en plaque, guttate, atrophoderma of Pasini and Pierini, keloidal, and lichen sclerosis et atrophicus: Generalized: Defined as involving 2 or more body areas: Bullous: Linear: Linear morphea of the extremities, en coup de sabre, and progressive facial hemiatrophy: Deep Dec 15, 2012 · Abstract. Intracranial abnormalities on computed tomography (CT) and magnetic resonance imaging (MRI) are present in a significant proportion of individuals with these conditions. 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. Linear morphea en coup de sabre (ECDS) is a form of localized scleroderma that predominantly affects the pediatric population, with a median age of 10 years at presentation. Current treatment paradigms rely on low levels of evidence, primarily 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. Linear Morphea occurs frequently in young children and manifests itself (when affected) as failure to grow one arm rapidly as expected. 3–5 ). Jul 6, 2023 · En coup de sabre - a deep-seated form of linear scleroderma affecting the face and scalp, presenting with an ivory sclerotic plaque, which evolves into a linear depressed grove. [ 3 ] Mar 14, 2018 · 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. 1 The pansclerotic plaques are ivory in color with hyperpigmented to violaceous 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. Some areas are dark while others are lighter than the surrounding normal skin. Over time the patches may become firm, dry and smooth. The authors performed three-dimensional reconstructions of MRI and CT scans to evaluate brain and bone structures. It typically occurs in children although cases of adult-onset ECDS exist as reported here. Linear scleroderma is most common in young girls. Sclerosis in ECDS lesions may invade deeply to involve Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. Apr 1, 2015 · Background Progressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology. 1% (71/109) were consistent with en coup de sabre lesions, and 31. J Am Acad Dermatol 2007;56:257-263. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy 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. 8 Tollefson, M. Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Due to the rarity of this Feb 1, 2017 · Abstract. 9 Eur J Intern Med. 3–4 ), or can be limited to the forehead or scalp, where is it known as en coup de sabre ( Fig. Linear morphea also has a higher rate of relapse compared with other variants. Sep 29, 2021 · Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. It can occur as a single lesion, usually on an extremity, can be widespread (Fig. Iran J Child Neurol. 2009 May;20(3):331-6. 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. djtybxdkhejuerpdoult