Morphea en coup de sabre If morphea crosses a joint, the thickening may limit joint movement. Headaches, uveitis and episcleritis are also sometimes present. However, through our study It usually affects children and young adults whom develop spontaneously, a scar-like band or plaque along the line of a limb or on the scalp - "en coup de sabre". Morphea is clinically divided into linear, circumscribed (plaque), generalized, pansclerotic, and mixed En coup de sabre (ECDS) is a rare variant of linear scleroderma typically found among children. There is loss of subcutaneous fat and the skin is tightly bound to the underlying sclerotic tissues creating a groove (en coup de sabre). We present a 26-year-old Caucasian female with a 1. J Am Acad Dermatol 2007;56:257–263. Google Scholar. 1,2 Morphea sites have been reported to coincide with those of other, pre-existing, inflammatory dermatoses, including herpes zoster. One subtype of morphea, en coup de sabre (ECS), is considered to be on a clinical spectrum with progressive facial hemiatrophy or Parry–Romberg syndrome (PRS) []. 2009 May;20(3):331-6. Classification of morphea (localized scleroderma). JH Lee, SY Lim, JH Lee, HC. 7 Although most cases present with a single lesion, multiple lesions can occur. Eosinophilic fasciitis , also known as Shulman syndrome, is often considered a part of the clinical spectrum of morphea. Epub 2008 Sep 17. Of the 82 cases of morphea identified in that study, 16 patients had linear scleroderma, including 4 with en coup de sabre and 2 with Parry-Romberg LS encompass 4 main categories: circumscribed morphea, generalized morphea, pansclerotic morphea and linear morphea, which is further subdivided into trunk/limb and head variants. (2014) Headaches as a presenting symptom of linear morphea en coup de sabre. En coup de sabre and Parry–Romberg syndrome are variants of linear morphea on Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Competing interests: None declared. There has been a great deal of discussion about the relationship of progressive facial hemiatrophy with localized scleroderma, but the coexistence of progressive facial hemiatrophy with lesions of linear scleroderma en coup de sabre or even with plaque morphea confirms that it is a variant of linear scleroderma. Further pro Other involvements that can occur are ocular and neurological; however, it is frequently associated with linear scleroderma, known as en coup de sabre. It distinguishes itself from diffuse forms of scleroderma, which often present with Raynaud’s phenomenon and visceral involvement []. Peterson L S, Nelson A M, Su W P D. Disease was present bilaterally in 7. 10. 1 Morphea en coup de sabre presents clinically in a band-like fashion over the frontoparietal region of scalp. PubMed. 1111/ijd. ECDS has a specific distribution on the frontal scalp and forehead and is usually unilateral. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic 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). had en coup de sabre morphea, 13 (24%) had Parry-Romberg syndrome, and 15 (28%) had both. Lewars DO Lehigh Valley Health Network, Marie_S. Pediatr Dermatol 17: 222-224 Morphea “en coup de sabre”: An unusual oral presentation Sven Niklander 1, Constanza Marín 2, René Martínez 3, Alfredo Esguep 3 1 DDS, MDent, MSc, Department of Oral Medicine and Oral Pathology, Universidad Andres Bello, Viña del Mar, Chile. J. The term en coup de sabre morphea refers to a lesion of linear morphea generally located in the frontoparietal scalp and/or the paramedian forehead, often resembling a stroke from a sword (). 4% of patients. Occasionally, involvement of underlying structures, including muscle, Linear morphea is the most common variant during childhood or adolescence; it begins at this stage in 40 to 70 % of patients. J Acad Dermatol. This diagnostic delay occurs because the clinical manifestations from the onset are polymorphic and not specific. We report a case of a 6-year-old female that presented with Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. It is an Scleroderma “en coup de sabre” (ECDS) is considered a linear localized form of scleroderma or morphoea. After thorough clinical, ultrasonography and brain Abstract: Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontopari-etal region of the head. Of patients who received treatment, most were treated with antimalarial agents (57. The aim of this article was to systematically analyze current data related to the treatment of 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. 5-year history of linear Background: The relationship between en coup de sabre morphea and Parry-Romberg syndrome is unclear, and not much is known regarding their demographic and clinical characteristics or the efficacy of the treatments that are used. Localized scleroderma can be classified as either circumscribed, linear, generalized, pansclerotic, or mixed variant morphea. 4317/jced. En coup de sabre morphea and Parry Romberg syndrome. En coup de sabre is a rare subtype of linear morphea that characteristically affects the face and En coup de sabre is a rare subtype of morphea. J Am Acad In children, linear morphea on the arms and legs can affect the underlying bone and interfere with bone growth. The histopathology of alopecia in linear morphea is typically characterized by sclerosis and a reduction in the number of follicular units. Acad. Figure 1 (A) A linear atrophic depression on the left forehead extending to the frontal scalp accompanied by cicatricial alopecia. This is the most frequent form of morphea of the scalp. In The en coup de sabre variant of linear scleroderma typically occurs in children. 10 While single lesions are most common, multiple lesions may coexist in a single patient, On the basis of a retrospective analysis of patients who developed morphea between 1960 and 1993 in Olmsted County Minnesota, the incidence of en coup de sabre is 0. This active pathological 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. Aydın H, Yologlu Z, Sargın H, Metin MR. 72–74 The disease particularly Background. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. Most pediatric patients have the linear subtype, which can extend deeply into the morphea en coup de sabre: thickened skin causing linear depressions on the forehead only (Find a localized scleroderma specialist at HSS. The words “progressive hemifacial atrophy,” “Parry–Romberg syndrome,” “localized scleroderma en coup de sabre,” “linear scleroderma en coup de sabre,” and “linear morphoea” were used for the search. It is usually treated with methotrexate and corticotherapy in addition to folic acid supplements. RF - rheumatoid factor. En Coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. We report a case of an elderly woman who 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. Due to the rarity of this condition, treatment is Morphoea (American spelling, morphea) is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. We diagnosed her as a case of linear scleroderma en coup de sabre (LSCS). 5). 8% Institutional review board approval was obtained for this study. 2007; 56: 257–63. Previously, it was Localized scleroderma/morphea en coup de sabre (LScs) is a rare form of localized scleroderma that typically affects predominantly children and women. Our study is the first to describe the surgical options and the outcomes of surgical interventions in patients with facial morphea. It began as an erythematous patch on the frontal scalp and forehead that 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. It can be mild, with only slight atrophy of the skin. En coup de sabre En coup de sabre (ECDS) manifests itself by the presence of linear atrophy and/or hardening of the skin, subcutis, muscles, and bones; it usually starts at the level of the upper eyebrow ridge and reaches the scalp, where a cicatricial alopecia focus appears [20, 21]. Physical, clinical, and imaging features. The early phase lesions appear as an erythematous or violaceous linear En coup de sabre is a rare entity that should be delineated from Parry-Romberg syndrome, as both entities share some common features and may coexist. En coup de sabre is a rare subtype of linear morphea that involves the frontoparietal scalp and forehead. Some include Parry–Romberg syndrome (PRS) or progressive hemifacial atrophy, a condition characterized by unilateral Ostertag JU et al. Morphea tends to have a waxing and waning course. 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. 1. 5 En coup de sabre is a rare subtype of morphea. It mainly affects the skin and subcutaneous tissue, resulting in cicatricial alopecia. Traditionally, it has been thought that the alopecia is not responsive to treatment and permanent. 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 (ECDS; “the cut of the sabre”) presents with an erythematous, sclerotic, atrophic linear plaque of morphea on the face, most commonly the paramedian forehead (Fig. It typically occurs in children although cases of adult-onset ECDS exist as reported here. 18-year-old female presenting with LM ECDS. J Dermatolog Treat 2018; 29(2): 197–199. Localized scleroderma (also known as morphea) is a chronic autoimmune disorder characterized by depressed, fibrotic, and dyschromic cutaneous lesions. One patient had a reduction in the size of two lesions (47% and 61% respectively) after 3-months follow-up, and the other patient had a reduction in lesion size of 42% after 21-months follow-up. Footnotes. Only patients with en coup Morphea en coup de sabre is a type of linear localized scleroderma, characterized by a sclerotic skin band in the forehaead and frontoparietal scalp area. 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, also known as scleroderma en coup de sabre, 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. En coup de sabre form of morphea often affects the scalp with thickening, sclerosis, dyspigmentation, and scarring alopecia. 5 Anderson LE, Treat JR, Licht DJ, Kreiger PA, Knight AM. It usually involves, unilaterally, the frontoparietal area, but may extend downwards to Hi odem my daughter was diagnosed with morphea en coup de sabre just over a month ago she is just 10 years old she has a dentation on her forehead she has finished her second round of steroids through IV and will have one more round next month for 3 days for 4 hours s day and on methotrexate for the next 3 years do you have any dentation on 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. We report a unique case of adult-onset en coup de sabre scleroderma in a patient with linear localized 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. 7. A person with a mild case develops a bit of tissue loss. 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 for linear scleroderma anywhere on the body) []. It began as an erythematous patch on the frontal scalp and forehead that 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. Here we describe 4 patients with ECDS who presented with headaches 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. Epilepsy, when present, is related to these brain lesions. (B) White fingernails with ground-glass opacities extending over nearly the entire nail bed resulting in an MORPHEA EN COUP DE SABRE AND PARRY–ROMBERG SYNDROME: WHEN IT IS WRITTEN ON THE FACE. Article PubMed CAS Google Scholar Dilley JJ, Perry HO (1968) Bilateral lineal scleroderma en coup de sabre. MAINTAINED ON SYSTEMIC THERAPY: A CASE PRESENTATION AND DISCUSSION Abstract Linear morphea in an en A patient with LS en coup de sabre at the Children's Hospital of Alabama was noted to have brain cavernomas by MRI. En coup de sabre is potentially the most disfiguring form of localized scleroderma, because it affects the face and scalp. Localized scleroderma differs from systemic scleroderma due to the absence of : Raynaud's phenomenon; acrosclerosis; systemic organ It usually affects children and young adults whom develop spontaneously, a scar-like band or plaque along the line of a limb or on the scalp - "en coup de sabre". It is possible that it is an Facial morphea linear lesions (eg, en coup de sabre lesions) may be associated with behavioral changes, learning disabilities and epilepsy. 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. She was a known case of linear morphea diagnosed by scalp biopsy, elsewhere. Linear scleroderma may coexist with progressive facial hemiatrophy (Parry “en coup de sabre”. Progressive hemifacial atrophy (PHA, Parry-Romberg syndrome) affects the face and head and may also affect the eye and brain [ 4 , 5 ]. Remission of seizures with immunosuppressive therapy in Parry-Romberg syndrome and en coup de sabre linear scleroderma: Case report and brief review Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Morphea is a sclerosing disorder of the skin and underlying tissues []. PM - plaque morphea. The lesion is usually unilateral and mostly affects the Tollefsen MM, Witman P. In literature, en coup de sabre morphea, and en coup de sabre scle-roderma are terms used interchangeably and synonymously. The relationship between en coup de sabre morphea and Parry-Romberg syndrome is unclear, and not much is known regarding their demographic and clinical characteristics or the efficacy of the Morphea (more-fee-uh): People see 1 or a few patches of thickened skin, which are usually red or purple. Thirteen percent of all patients in the study group had seizures. Corticosteroids remain the treatment of choice, but other modalities such as methotrexate, excimer laser, and grafting have been used with varying success. Am. Neurosciences Of those with linear lesions located on the head or neck (N = 109), 65. Imaging findings and histopathology indicated that the process, most likely focal inflammatory, may be progr Linear morphea en coup de sabre is a localized form of morphea that presents as paramedian face or frontal scalp depression. Methotrexate is the first-line treatment for LM, although systemic steroids, topical agents (corticosteroids and calcineurin 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. En coup de sabre (linear atrophic band +/- sclerosis, classically on the forehead or scalp) 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. The diagnosis of morphea en coup de sabre is most often made at an advanced pathological stage. Morphea, also known as localized scleroderma, is a rare disease seen in both adults and children. 2% (34/109) had Parry-Romberg syndrome, also known as hemifacial atrophy. Due to the rarity of this condition, treatment is Abstract. (2000) Bilateral en coup de sabre-a rare entity. Only a few bilateral cases have been reported to date. Lewars@lvhn. 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. en coup de sabre,linear scleroderma, morphea The term“en coup de sabre”is used for linear scleroderma located in the temporoparietal region that produces sharp bordered furrowing with alopecia when extending to the scalp. e. comments sorted by Best Top New Controversial Q&A Add a Comment. ECDS - en coup de sabre. . En coup de sabre is a rare subtype of morphea. Diese beginnt meist mit einem kleinen roten ovalen Fleck (Erythem) an den Extremitäten oder am Stamm, der sich schleichend vergrößert. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic lesions over the forehead with 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 Linear morphea involving the frontoparietal region presents as a densely sclerotic plaque extending from the eyebrow to the scalp. Rai R et al. Of note, 13. 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. We describe her ocular, adnexal and tomographic Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions are inherently different processes or appear on a MORPHEA EN COUP DE SABRE AND PARRY–ROMBERG SYNDROME: WHEN IT IS WRITTEN ON THE FACE. Although ECDS tends to start on the scalp, this line may extend downwards to involve inferior areas such as En coup de sabre (morphea) experience? Hi guys, Does anybody here have the linear scleroderma type, specifically 'en coup de sabre' on their head? I'm looking for someone to share experiences with, help me out. , Witman, P. Dermatology 45: 398-401; Polcari I et al. Arch Dermatol 97: 688–689. Morphea is distinct from systemic sclerosis (scleroderma), percent; Generalized ‒ 8 percent; Linear ‒ 6 percent; En coup de sabre (a form of linear disease) ‒ 3. morphoea en coup de sabre had lesion size measured at baseline and again after starting abatacept treatment. The 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. 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. Background: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. Key words: linear morphea en coup de sabre (LM ECDS), scleroderma, Parry-Romberg syndrome (PRS) FIGURE 1. It can cause cicatricial alopecia, when En coup de sabre is a rare subtype of morphea. 9 Eur J Intern Med. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic lesions over the forehead with Morphea can be classified into 5 main types (limited, generalized, linear, deep, and mixed), as well as several clinical subtypes (eg, plaque-type, pansclerotic, linear, en coup de sabre). 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. Sclerosis in ECDS lesions may invade deeply to involve underlying Distinctive histopathologic findings in linear morphea (en coup de sabre) alopecia. Objective: The purpose of this study was to describe demographic data, clinical features, and medications used in a large cohort of patients with en Linear scleroderma en coup de sabre is a rare chronic autoimmune disease. 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. Current treatment paradigms rely on low levels of evidence, primarily case Morphea is a skin condition that involves a patch or patches of discolored or hardened skin on the face, neck, hands, torso, or feet. Niklander and Constanza Marin and Ren{\'e} Martinez and Alfredo Esguep}, journal={Journal of Clinical and Experimental Dentistry}, year={2017}, Morphea Die häufigste Form ist die limitierte LS – die Plaque-Form (auch Morphea genannt). The hair is lost permanently and the underlying skull 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. J Neurol Neurosurg Psychiatry. In certain cases, skin lesions extend downwards to the nose, cheek, upper lip, and involve the mouth High potency topical steroids and calcineurin inhibitors were prescribed along with photoprotection. 5. Pediatrics 134: e1715-1719 . Hautarzt 45: 398-401; Polcari I et al. This form of localized morphea is most commonly En coup de sabre (ECDS) is a form of linear scleroderma or morphea that distinctly appears on the forehead and/or frontoparietal scalp. Pediatr Dermatol 17: 222-224 Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup Linear scleroderma, also known as scleroderma en coup de sabre, 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. Hooman Khorasani, a quadruple board-certified surgeon, repairs an atrophic scar as the result of linear scleroderma aka Morphoea en coup de sabr However, linear morphea, including the en coup de sabre subtype, may remit and reactivate, remain unchanged, or become more extensive with time. At 4-month follow-up, all his lesions had mostly cleared. It manifests by presence of linear atrophy and/or hardening of the skin, subcutis, occasionally involving muscles and bones. However, there are no case series using methotrexate treatment for En coup de sabre. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. ImmaFighter • The original articles, reviews, and cases from Medline PubMed were the main source of information. LS - localised scleroderma. This is referred to as en coup de sabre because it makes 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. Crossref. Because of its occurrence in young populations and its very visible location, en coup de sabre is a socially stigmatizing pathological 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. Article Google Scholar Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. 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. It has a significant impact on the patient’s daily life due to the unaesthetic evolution of the cutaneous lesions. En coup de sabre is a subtype of linear morphea (localized scleroderma) localized to the paramedian forehead or frontoparietal scalp and may involve subcutaneous tissue, muscle, and bone. 197-199. Start your 7-day free trial to receive access to high fidelity premium pronunciations. En coup de sabre is a localized variant of scleroderma that presents as a linear, atrophic depression affecting the frontoparietal aspect of the face and scalp. The lesion is usually unilateral and mostly affects the Morphea “en coup de sabre” (MCS) is a type of linear morphea that can involve the frontal and parietal region of the scalp, and the face. Intracranial abnormalities on computed tomography (CT) and magnetic resonance imaging Morphea en coup de sabre (51156002); Coup de sabre scleroderma (51156002) Definition. Linear morphea also has a higher rate of relapse compared with other variants. The head variant termed “en coup de sabre” (LSES) is characterized by an indurated long streak resembling the deep wound of a sword. Brain MRI and head CT (i. Due Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. En coup de sabre morphea treated with hyaluronic acid filler. References. ECS involves the face or scalp; PRS results in progressive, self-limited facial hemiatrophy. Unlock premium audio pronunciations. We report a case of a 12-year-old male child with two linear brownish depressed asymptomatic DOI: 10. 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 OL39šÛßlâ&„IÔU* iÄŠq ‰%¦‚V–ZÀS ñíŠ †g2bØèµ&¹ \yÉ–Ê Ûã·öo¹[ þ¶ùÓt Á¶i» v]Ó° ‹°†±å: F Ñl‚=Çñ°f ®E0F–f –‹1r ÍÄži [3L ›  ±] !ϳ5 {¦ ˆå¹¦F\Ó´ âj:Á¦c:–iY 6LâaÓ²5Û² [#®«y " Ñ0q‰æ"× 66] &ÁŽë Ò ¦cä ;–ak–i ×" Ñ°kXȶlM7mÇDıˆf How do you say Morphea en coup de sabre? Listen to the audio pronunciation of Morphea en coup de sabre on pronouncekiwi. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. 2. 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). Report of a case and review of the literature Int J Dermatol. En coup de sabre can Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. 7,12,13 It manifests as a solitary, linear, fibrous plaque that involves the skin, underlying muscle, and bone. This report presents two cases with extensive, apparent scarring alopecia that improved with medical treatment. 2007;56:257–63. We present a case of an 8-year-old girl with a left frontoparietal “en coup de sabre” scalp A case of a 42-year-old female treated with hyaluronic acid filler while maintained on systemic therapy to prevent disease progression is reported and Dermal fillers provide a cosmetic option that can be used in conjunction with systemic therapies. J Cutan Pathol (2013) 40 (6):580–4. This Morphea (localized scleroderma) in adults: Management Chung MH, Sum JM, Morrell MJ, Houpian DS (1995) Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings. Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. 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. Arch Craniofac Surg, 18 (2017), pp. 15. However, depending on its locations on the face, it can lead to considerable problems, especially in children. Ostertag JU et al (1994) Bilateral linear tempoparietal scleroderma en coup de sabre. Watch as Dr. In 1854, Addison coined the term ‘en coup de sabre’ as the lesion resembled the scar resulting from a saber (sword) cut. 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. Current treatment paradigms rely on low levels of evidence, primarily case En coup de sabre ('cut from a sword' in French) is a deep seated form of the linear scleroderma affecting the scalp and face like a sabre cut. We describe a case of linear scleroderma en coup de A 70-year-old woman presented with a 1-year history of progressive indentation and darkening of the skin of her forehead. The histopathology of alopecia in linear morphea is typically . The efficacy of methotrexate in the treatment of en coup de sabre (linear morphea subtype). doi: 10. The existence of neurologic findings in association with ECDS has been well described in the literature. In 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. A novel case of en coup de sabre is reported in which hyaluronic acid filler alone was successfully used to correct the atrophic defect. 12124 [Google Scholar] 64. Dermatol. This disorder can affect the tissues under or near the lesion including brain, bone and eyes. Linear morphea is the most common subtype of morphea in children. Linear scleroderma may coexist with progressive facial hemiatrophy (Parry Linear morphea en coup de sabre is a localized form of morphea that presents as paramedian face or frontal scalp depression. 13 cases per 100,000 population . Homayoon D, Haybaeck J, Aberer E. JLS - juvenile localised scleroderma. Linear scleroderma “en coup de sabre” coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids. Adult morphea en coup de sabre with accompanying regional polymyositis: a separate entity. Parry-Romberg syndrome. In addition, patients with linear lesions may develop limb atrophy and contractures that result in limited movement and En coup de sabre is a rare subtype of morphea. 1111/cup. 12108. Crossref View in Scopus Google Scholar. The patches can itch but are usually painless. 166-171. Linear morphea on the head (also called en coup de sabre) may extend inwards to the brain and cause seizures. On examination, there was a linear depression on the forehead that extended Based on these findings, Terry's nail and morphea en coup de sabre associated with MCTD were diagnosed. En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. The Abstract. Introduction. Ann Neurol 37: 679–681. Institutional review board approval was obtained for this study. Image courtesy of: Josette McMichael, MD; Atlanta, Georgia, USA. Here we describe 4 patients with ECDS who presented with headaches, which were Morphea en coup de sabre is a rare pat hology that causes significant aestheti c damage to the face and for which, to date, no curative treatment has been found yet. 1 It may extend down the face as well. (1994) Bilaterale lineare tempoparietale Sklerodermie en coup de sabre. 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. En coup de sabre: A line of thickened skin forms on the scalp, face, or both, and the tissue beneath disappears. ) 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. Ahn. Report of a case and review of the literature. Surgical Management of Localized Scleroderma. Current attempts for Morphea en coup de sabre (ECDS) is a type of linear morphea that involves the head and scalp. 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. Only patients with en coup Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. Characterized by excessive collagen deposition, the disease leads to sclerosis and subsequent atrophy. Intracranial Linear scleroderma, also known as scleroderma en coup de sabre, is a very focal form of scleroderma classically characterized by a linear band of atrophy involving the frontal Autologous fat grafting (Coleman's technique) offers a safe and simple approach for the treatment of linear scleroderma "en coups de sabre". 1% (71/109) were consistent with en coup de sabre lesions, and 31. However, in about 32% of cases, it may arise in adulthood, showing a more aggressive course with also an increased risk of systemic involvement. Objective: To evaluate the efficacy and Morphea en coup de sabre is a form of linear scleroderma that primarily affects the head and neck and is named from its linear shape and characteristic lesions that resemble the wound from a saber. In literature, en coup de sabre morphea, and en coup de sabre scleroderma are terms used interchangeably and synonymously. 1%) or methotrexate En Coup de Sabre Marie S. Systemic treatment of localised morphea with methotrexate has been reported in a few clinical reports. 1 . org Gwyn Frambach DO 8 Tollefson, M. Oral involvement is unusual and it Unterberger I, Trinka E, Engelhardt K, et al. Localized scleroderma differs from systemic scleroderma due to the absence of : Raynaud's phenomenon; acrosclerosis; systemic organ 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. It is more common in females than in males (2). We report here the first case, to our knowledge, of discoid lupus erythematosus with en coup de saber presentation mimicking morphea. The efficacy of methotrexate in the treatment of en coup de sabre (linear morphea subtype) J Dermatolog Treat, 29 (2018), pp. Intracranial abnormalities on computed tomography (CT) and magnetic resonance imaging (MRI) are present in a significant proportion of i Linear morphea en coupe de sabre (LM) usually arises in childhood. 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. 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 Scleroderma is a rare connective tissue disorder categorized into systemic sclerosis and localized scleroderma, also called morphea. Although it is a benign disease,the resultant marked deformity often necessitates treatment. as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. It is accompanied by loss of adnexal structures, pruritus and xerosis. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. J Am Acad Dermatol 2007;56:257-263. GM - generalised morphea. A retrospective review of 54 patients. 11 It mainly affects the extremities, the head in the frontal area (morphea en coup de sabre) and the chest. Current attempts for The term en coup de sabre morphea refers to a lesion of linear morphea generally located in the frontoparietal scalp and/or the paramedian forehead, often resembling a stroke from a sword . Die Sklerodermie en coup de sabre ("Säbelhieb-Sklerodermie") ist eine Sonderform und tritt meist im Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. The patients with deeper lesions can develop ocular and neurologic symptoms 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). In general 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. 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. 8 The exact etiology of this disease remains to 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 All patients had craniofacial scleroderma: linear scleroderma en coup de sabre (LScs), progressive facial hemiatrophy (PFH, or Parry-Romberg syndrome) or both. en coup de sabre or progressive facial hemiatrophy): Detect muscle, joint or bone involvement Detect potential subcortical calcifications or brain atrophy Morphea profunda, morphea of childhood, generalized morphea, linear morphea Panniculitis is predominantly septal, resulting in widening and thickening Reconstruction of the face and scalp may be beneficial to patients with en coup de sabre and Parry-Romberg syndrome, with possible use of tissue expansion and implants of autologous bone, fat, or synthetic materials (eg, polyethylene). A type of linear scleroderma characterized by a linear, colorless, atrophied band across the forehead or scalp. 2003; 74: 661–664. 2015 Jul;54(7):823-6. itefo wkka hfgi fpeajf pazpb dhuqalp rlkbk arbqxiw fddrli bzyc