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Morphea in coup sabre

Karan Seegobin, Kamille Abdool, Kanterpersad Ramcharan, Haramnauth Dyaanand, Fidel Rampersad
We describe a case of Parry Romberg syndrome/en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy...
September 30, 2016: Neurology International
Muhammad Amjad Khan, Lindsay Shaw, Despina Eleftheriou, Prab Prabhakar, Wui K Chong, Mary Glover
We report the case of a young girl who presented with hemiparesis, seizures, and subtle features consistent with a linear form of facial morphea (en coup de sabre). She was treated with pulsed parenteral steroids and oral steroids and started on methotrexate. Magnetic resonance imaging results and neurologic problems improved after 6 months. Switching off inflammation early in the course of disease seemed to reverse some of the central nervous system changes. Assessment of children with unexplained hemiparesis and seizures should include careful examination of the face and scalp, looking for subtle signs of skin change and asymmetry...
March 2016: Pediatric Dermatology
Leelavathy Ganesan, Heena Parmar, Jayanta Kr Das, Asok Gangopadhyay
Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis with anogenital and extragenital presentations. Extragenital lichen sclerosus is most common on the neck, shoulders and upper trunk. Linear lesions are uncommon in LSA. We report a case of linear extragenital LSA involving forehead and scalp, along with grouped white papules of LSA in the right side of the back in a postmenopausal woman. The patient showed atypical clinical presentation of LSA in face which clinically mimicked 'en coup de sabre' as seen in morphea, but other clinical features suggested the diagnosis of LSA and the histopathological findings confirmed it...
July 2015: Indian Journal of Dermatology
Stanislav N Tolkachjov, Nirav G Patel, Megha M Tollefson
BACKGROUND: Progressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology. The true incidence of this disorder has not been reported, but it is often regarded as a subtype of localized scleroderma. 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 spectrum (; Adv Exp Med Biol 455:101-4, 1999; J Eur Acad Dermatol Venereol 19:403-4, 2005)...
2015: Orphanet Journal of Rare Diseases
Dominique Van der Veken, Petra De Haes, Esther Hauben, Wim Teughels, Paul Lambrechts
Morphea is an inflammatory disorder of the skin and underlying tissues characterized by an overabundance of collagen leading to fibrosis. The prevalence of this disease is estimated at around 0.4-2.7/100,000 people. When the process occurs in the gingival tissues, it induces traction, which can cause gingival recession. A 19-year-old woman was referred to the clinic for a progressive recession on teeth 11 and 12. A pale, atrophic, linear region extending from her nose to her upper lip on the right-hand side of her face was diagnosed as morphea en coup de sabre...
May 2015: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Ingrid Polcari, Amanda Moon, Erin F Mathes, Elaine S Gilmore, Amy S Paller
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 existence of neurologic findings in association with ECDS has been well described in the literature. Here we describe 4 patients with ECDS who presented with headaches, which were typical migraines in 3 of the patients. The headaches preceded the onset of cutaneous findings by at least 6 months. Our patients' cases emphasize both the importance of recognizing headaches as a harbinger of ECDS and the necessity of performing thorough cutaneous examination in patients with unexplained headaches or other neurologic disease...
December 2014: Pediatrics
Derrick A Doolittle, Vance T Lehman, Kara M Schwartz, Lily C Wong-Kisiel, Julia S Lehman, Megha M Tollefson
INTRODUCTION: Parry-Romberg syndrome (PRS) and en coup de sabre (ECS) are variants of morphea. Although numerous findings on central nervous system (CNS) imaging of PRS and ECS have been reported, the spectrum and frequency of CNS imaging findings and relation to cutaneous and neurologic abnormalities have not been fully characterized. METHODS: We retrospectively reviewed patients younger than 50 years at our institution over a 16-year interval who had clinical diagnosis of PRS and ECS by a skin or facial subspecialist...
January 2015: Neuroradiology
N Cassano, P Amerio, R D'Ovidio, G A Vena
Hair disorders are frequently observed in various systemic diseases, including autoimmune connective tissue diseases (CTDs), with predilection of lupus erythematosus (LE), followed by dermatomyositis (DM) and scleroderma. Hair disorders in CTDs may manifest as various clinical patterns, such as telogen hair loss, diffuse thinning or fragility of hair, and scarring alopecia. Less common hair disorders include anagen effluvium, alopecia areata, and trichomegaly. Some drugs used to treat CTDs may cause hair loss in a drug-related manner or hyperthrichosis...
October 2014: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
A Parodi, E Cozzani
Hair loss is commonly seen in autoimmune diseases. In pemphigus, although scalp involvement is common, hair loss is rarely reported. In classical bullous pemphigoid, alopecia is not reported while it is described in the Brusting-Perry variant of bullous pemphigoid and in epidermolysis bullosa acquisita. In these two diseases alopecia is cicatricial. In connective tissue diseases, in lupus erythematosus (LE) hair loss is frequent; in particular in LE there are two types of alopecia: non scarring and scarring alopecia...
February 2014: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Claudia Gutiérrez-Gómez, Ana L Godínez-Hana, Marisela García-Hernández, María de Lourdes Suárez-Roa, Sonia Toussaint-Caire, Elisa Vega-Memije, Daniela Gutiérrez-Mendoza, Marcia Pérez-Dosal, Carlos E Medina-De la Garza
BACKGROUND: Progressive hemifacial atrophy or Parry-Romberg Syndrome (PRS) is a rare, acquired, progressive dysplasia of subcutaneous tissue and bone characterized by unilateral facial involvement. Its etiology is unknown, but theories about its pathogenesis include infectious, degenerative, autoimmune, and traumatic causes among others. The causal relationship of PRS and linear morphea en coup de sabre (LMCS) with Borrelia burgdorferi infection remains controversial. Our goal was to serologically determine anti-B...
August 2014: International Journal of Dermatology
Chetan Vedvyas, Richard W Urbanek
We report the case of a 44-year-old woman with a one-year history of en coup de sabre morphea and progressive hemifacial atrophy with ipsilateral hemifacial neuralgia, migraine, and contralateral neurologic abnormalities. While rare, Parry-Romberg syndrome typically presents in the first or second decade of life; this patient's case is unusual in that the onset of her disease is demonstrated at age 43. Common clinical features, laboratory findings, and pathogenetic theories are discussed.
December 2013: Dermatology Online Journal
Margareth Pierre-Louis, Leonard C Sperling, Mark S Wilke, Maria K Hordinsky
Linear morphea en coup de sabre is a localized form of morphea that presents as paramedian face or frontal scalp depression. The histopathology of alopecia in linear morphea is typically characterized by sclerosis and a reduction in the number of follicular units. We present a 26-year-old Caucasian female with a 1.5-year history of linear morphea and alopecia with unique atrophic follicular remnants on scalp biopsy. Transverse and vertical sections of biopsy specimens showed dense, dermal sclerosis with marked eccrine gland atrophy and replacement of much of the adipose by collagenous tissue...
June 2013: Journal of Cutaneous Pathology
Kristen Miller, Stephanie Lehrhoff, Max Fischer, Shane Meehan, Jo-Ann Latkowski
Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. It typically occurs in children although cases of adult-onset ECDS exist as reported here. ECDS has a specific distribution on the frontal scalp and forehead and is usually unilateral. Sclerosis in ECDS lesions may invade deeply to involve underlying muscle and bone and may exist on the same clinicopathologic spectrum as Parry-Romberg syndrome. Extracutaneous involvement is frequent, with neurologic and ophthalmologic findings occurring most commonly...
December 2012: Dermatology Online Journal
Yvonne E Chiu, Sheetal Vora, Eun-Kyung M Kwon, Mohit Maheshwari
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. Intracranial abnormalities on computed tomography (CT) and magnetic resonance imaging (MRI) are present in a significant proportion of individuals with these conditions. We describe 32 children from our institution with ECDS or PRS; neuroimaging was performed in 21 cases. We also review 51 additional cases from the literature. Nineteen percent of the children at our institution with ECDS or PRS had intracranial abnormalities on MRI, half of whom were asymptomatic...
November 2012: Pediatric Dermatology
Paweł Pietkiewicz, Justyna Gornowicz-Porowska, Monika Bowszyc-Dmochowska, Marian Dmochowski
The commonest source of naturally acquired microchimerism, i.e. small numbers of foreign cells within the organism, is two-way mother-fetus transplacental trafficking during pregnancy. Here, the first report on coexistence of pregnancy-associated pemphigoid gestationis (PG) and progressive facial hemiatrophy, a form of "en coup de sabre" morphea, is presented. HE histopathology (eosinophil-rich subepidermal infiltration, inverted teardrop sign), direct immunofluorescence (linear IgG1, but not IgG4, deposits along the dermal-epidermal junction) and ELISA (elevated levels of serum and blister fluid IgG autoantibodies to BP180) corroborated the PG diagnosis...
March 2012: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
Carolyn Goh, Asok Biswas, Lynne J Goldberg
Linear scleroderma en coup de sabre ('the stroke of the sword') is an uncommon form of morphea with onset typically in childhood or adolescence. Involvement is usually located on the paramedian forehead and is associated with alopecia. It is microscopically indistinguishable from other forms of scleroderma. We present a 51-year-old woman who presented with alopecia and subsequently developed linear scleroderma en coup de sabre on her adjacent forehead. Histopathology revealed a strikingly perineural lymphocytic and plasmacytic infiltrate, extending deeply into the subcutis and fascia...
May 2012: Journal of Cutaneous Pathology
Elhem Khelifa, Isabelle Masouye, Hoang Chinh Pham, Laurent Parmentier, Luca Borradori
BACKGROUND: Overlap syndromes represent disorders that combine diagnostic criteria of two or more different connective tissue diseases. METHODS: We herein describe the case of a 34-year-old patient. RESULTS: Our patient developed a lesion on the scalp and forehead following Blaschko's line typical for linear morphea "en coup de sabre", while histopathological features were consistent with both chronic cutaneous lupus erythematosus and linear morphea, a cutaneous overlap syndrome previously described as linear sclerodermiform lupus erythematosus...
December 2011: International Journal of Dermatology
Elizabeth Lazaridou, Christina Giannopoulou, Zoi Apalla, Christina Fotiadou, Anastasia Trigoni, Demetris Ioannides
BACKGROUND: Parry-Romberg syndrome (PRS) or idiopathic hemifacial atrophy is a rare neurocutaneous syndrome. It is characterized by slowly progressive atrophy, located on one side of the face, primarily involving the skin, fat and connective tissue. PRS seems to overlap with "en coupe de sabre" morphea. MAIN OBSERVATIONS: We present a case of hemifacial atrophy in a 14-year-old boy treated with topical calcipotriol-betamethasone ointment. The diagnosis of PRS was established mainly based on the clinical findings and histological picture...
November 19, 2010: Journal of Dermatological Case Reports
Jelena Maletic, Vassiliki Tsirka, Panos Ioannides, Dimitrios Karacostas, Nikolaos Taskos
Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma 'en coup de sabre'. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea)...
2010: Case Reports in Neurology
Nadia El Fékih, Iménc Réjaibi, Hajer Kamoun, Faten Zéglaoui, Bécima Fazaa, Monia Kharfi, Mohamed Ridha Kamoun
BACKGROUND: Sclerodermas are rare affections which can be located or generalized. Localized form is the most frequent. AIM: The purpose of this study was to describe epidemiologic, clinics, biological, immunological, therapeutic, evolutionary characteristics of the localized scleroderma through a personal series and the data of the literature. METHOD: We have performed a retrospective study on all patients followed in the department of dermatology of the Hospital Charles Nicole during 14 years period...
September 2009: La Tunisie Médicale
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