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Parry Romberg AND morphea

Mark Mansour, Carmen Liy Wong, Francesco Zulian, Suzanne Li, Kimberly Morishita, Eluen Ann Yeh, Katie Stewart, Ronald M Laxer, Elena Pope
BACKGROUND: Congenital morphea is a form of localized scleroderma that presents at birth. There is limited information on its presentation and progression. METHODS: Patients with congenital morphea were identified from five pediatric dermatology and rheumatology tertiary care centers in Canada, the United States, and Italy from 2001 to 2016. Cases from the literature were identified by searching Ovid (EMBASE and MEDLINE) from inception to June 30, 2017. Disease characteristics and prevalence of extracutaneous involvement were analyzed...
November 2018: Pediatric Dermatology
Ikram E Duman, Gazanfer Ekinci
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. 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. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. We present a case of an 8-year-old girl with a left frontoparietal "en coup de sabre" scalp lesion and describe the neuroimaging findings of frontoparietal white matter lesion discovered incidentally on routine magnetic resonance imaging...
June 2018: Radiology Case Reports
Hatice Ataş, Müzeyyen Gönül, Aysun Gökçe, Mutlu Acar, Canan Gürdal
Parry-Romberg syndrome (PRS) may overlap localized scleroderma (morphea) lesions with linear depression (en coup de sabre [ECDS]). Overlap case with PRS and ECDS was presented. Enophthalmos, uveitis, ocular torticollis, keratic linear precipitates, and anti-double-stranded DNA positivity were identified. Subendothelial keratic precipitates detected by an in vivo laser scanning confocal microscopy were the first profiled in the literature. Patients must be evaluated and followed up carefully by their clinics to prevent misdiagnosis and unnecessary procedures such as surgery of ocular torticollis as muscular torticollis...
February 2018: Indian Journal of Ophthalmology
Shantanu Dixit, Chaithra Kalkur, Atul P Sattur, Michael M Bornstein, Fred Melton
BACKGROUND: Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable...
October 24, 2016: Journal of Medical Case Reports
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
Hisashi Nomura, Shohei Egami, Tomoaki Yokoyama, Makoto Sugiura
A lot of diseases, including lupus profundus, morphea, lipodystrophy, and Parry-Romberg syndrome, may manifest progressive hemifacial atrophy. These diseases usually progress slowly and rapid progression of atrophy is extremely rare. We report a case of elderly-onset rapid progression of hemifacial atrophy only in three weeks. Our case did not meet variable differential diagnoses. We discuss the clinical character of the patient against the past of literature and suppose it may be a new clinical entity.
2015: Case Reports in Dermatological Medicine
Justine Vix, Stéphane Mathis, Mathieu Lacoste, Rémy Guillevin, Jean-Philippe Neau
Parry-Romberg syndrome (PRS) is a variant of morphea usually characterized by a slowly progressive course. Clinical and radiological involvement of the central nervous system may be observed in PRS. We describe 2 patients with PRS and neurological symptoms (one with trigeminal neuralgia associated with deafness, and the second with hemifacial pain associated with migraine without aura) in conjunction with abnormal cerebral MRI including white matter T2 hyperintensities and enhancement with gadolinium. Despite the absence of specific immunosuppressive treatments, both patients have presented stable imaging during follow-up without any clinical neurologic progression...
July 2015: Medicine (Baltimore)
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)...
April 1, 2015: Orphanet Journal of Rare Diseases
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
Pragya A Nair, Nidhi H Patel, Nilofar G Diwan
Parry-Romberg syndrome is a rare neurocutaneous syndrome characterized by progressive shrinkage and degeneration of the tissues beneath the skin, usually on one side of the face. It is associated with neurological disorders such as trigeminal neuralgia, facial paresthesia, headache, and focal epilepsy. Concomitant occurrence of discoid lupus erythematosus (DLE) and morphea in the same skin lesion is exceptional, defined as overlap syndrome with two or more different connective tissue disease concurrently or consecutively...
April 2014: International Journal of Trichology
Mohsin Khan, Mubeen Khan, Raju Negi, Nikita Gupta
Parry Romberg syndrome(PRS) is a rare acquired poorly understood neurocutaneous syndrome of unknown etiology characterized by slow progressive atrophic changes commonly affecting one half of the face. The exact incidence and etiology towards the syndrome remains unclear. Apart from the multifactorial etiology proposed, the possible primary cause is mainly attributed to the cerebral disturbance of the fat metabolism. The syndrome overlaps with "en coup de sabre" morphea, with an ill defined relationship existing between the two...
July 2014: Journal of Clinical and Experimental Dentistry
Claudia Saad Magalhães, Taciana de Albuquerque Pedrosa Fernandes, Thiago Dias Fernandes, Luis Antonio de Lima Resende
BACKGROUND: Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS. METHODS: We conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997-2013...
2014: Pediatric Rheumatology Online Journal
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
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
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
Ximena Wortsman, Jacobo Wortsman, Ivo Sazunic, Laura Carreño
BACKGROUND: Morphea (circumcripted cutaneous scleroderma) can be difficult to assess for lesion activity. Because variable-frequency ultrasound with color Doppler provides details of skin morphology and function, it may help in the categorization of morphea. OBJECTIVE: We sought to evaluate color Doppler ultrasound as a probing tool for assessing activity in morphea lesions. METHODS: Consecutive patients with cutaneous morphea referred by dermatologists were studied with color Doppler ultrasound, and the assessment of lesion activity was compared with histologic findings...
November 2011: Journal of the American Academy of Dermatology
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
Tameka K Lane, Jessie Cheung, Julie V Schaffer
We present a 14-year-old girl with a nine-year history of progressive subcutaneous atrophy on the right side of the face, which is consistent with a diagnosis of Parry-Romberg syndrome. The onset of the atrophy was heralded by the development of a morpheaform plaque on the right temple. This case highlights the substantial of overlap of Parry-Romberg syndrome and morphea.
October 15, 2008: Dermatology Online Journal
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