scholarly journals Parry Romberg Syndrome with localized scleroderma: a case report

Author(s):  
M. Khan ◽  
M. Khan ◽  
R. Neg ◽  
N. Gupta
2019 ◽  
Vol 25 (3) ◽  
pp. 28
Author(s):  
Alexandre Puidupin ◽  
Aline Desoutter ◽  
Sophie Deneuve ◽  
Lara Nokovitch ◽  
Anne-Gaëlle Chaux-Bodard

Introduction: The Parry Romberg syndrome (PRS) is a mosaic disease of unknown aetiology which mostly affects women. The facial hemiatrophy generally begins during the early childhood. It has a great impact on social life, and aesthetic rehabilitation is a major challenge. Observation: A 38 years-old-female patient presented with an enucleation of the left eye, due to multiple hamartomas and progressive facial hemiatrophy. The placement of 2 extraoral implants was proposed to bear an ocular epithesis. Comments: PRS develops between the 2nd and 20th year of life, and stabilizes at the adult age. Its main characteristic is a progressive facial hemiatrophy, which involves skin, fat tissues, muscles and osteocartilaginous tissues. 20% of patients have neurological symptoms, and epilepsy is often described as a side effect of the disease. Conclusion: Diagnosis of localized scleroderma has to be eliminated, as these 2 entities are very similar. Major facial reconstructive surgery is often proposed to restore aesthetics.


2013 ◽  
Vol 4 (2) ◽  
pp. 68-71
Author(s):  
Ehsanul Haque Khan ◽  
MA Rashid ◽  
Taslima Hoq Moonmoon ◽  
Monzur Ahmed ◽  
Bidoura Tanim

Parry-Romberg Syndrome is an uncommon, degenerative and poorly understood condition. It is characterized by a slow and progressive atrophy affecting one side of the face. Possible factors that are involved in the pathogenesis include trauma, viral infections, heredity, endocrine disturbances and auto-immunity. Characteristically the atrophy progresses slowly for several years and soon after it become stable. In this case report, the patient of 8 years old girl complaints of gradual wasting of left side of face for 1 year involving cheek, chin, pre-auricular area, forehead, left upper lip which reveals facial asymmetry and mild wasting of left side of face as well as tongue. There was focal alopecia and depression over left side of scalp. After four months back, there was a hypopigmented area over malar prominence of same side. She was diagnosed as Parry Romberg syndrome on the basis of history, clinical examination and investigation. Antinuclear antibody and Anti-Scl-70 antibody were positive. Histopathological diagnosis was compatible with localized scleroderma. The patient was treated with drugs, physical therapy including electric stimulation as well as exercise. DOI: http://dx.doi.org/10.3329/jssmc.v4i2.14424 J Shaheed Suhrawardy Med Coll, 2012;4(2):68-71


Author(s):  
Giacomo Evangelista ◽  
Fedele Dono ◽  
Claudia Carrarini ◽  
Mirella Russo ◽  
Laura Bonanni

2017 ◽  
Vol 23 (4) ◽  
pp. 370-373
Author(s):  
P Alirezaei ◽  
L Khezrian ◽  
H Ghasemi Basir ◽  
S Khanali ◽  
◽  
...  

2017 ◽  
Vol 45 ◽  
pp. 26-30
Author(s):  
Guiomar Fernández-Castellano ◽  
Rosana Guerrero-Domíinguez ◽  
Daniel López-Herrera-Rodríguez ◽  
Ignacio Jiméenez

2020 ◽  
Vol 35 (11) ◽  
pp. 753-762
Author(s):  
Ronald R. Seese ◽  
Daniel Glaser ◽  
Andre Furtado ◽  
Kavita Thakkar ◽  
Kathryn S. Torok

Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Methods: Neuroimaging and neurological symptoms of children evaluated at our institution with a diagnosis of PRS or ECDS were retrospectively reviewed. Laterality, location, stability, and number of lesion(s) were evaluated, as was the presence of susceptibility lesion(s) and contrast enhancement. History of seizures or headaches was noted. Results: From 2003 to 2019, 80 patients with PRS or ECDS were followed at our institution. Neuroimaging was completed in 73 and found to be abnormal in 25. In 12 (48%) of these 25 cases, headaches and/or seizures were present. In the vast majority of these cases (22/25, 88%), lesions were ipsilateral to skin findings. White matter was involved in 19 (76%) patients. MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial compartment, did not enhance, and were stable at 1-year follow up imaging. Of those with progression, susceptibility findings were present at baseline. Conclusions: Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous findings.


2010 ◽  
Vol 34 (2) ◽  
pp. 127-130
Author(s):  
Ian C. Hoppe ◽  
Matthew J. Trovato ◽  
Gregory E. Rauscher

1993 ◽  
Vol 36 (3) ◽  
pp. 410-415 ◽  
Author(s):  
Nina Birdi ◽  
Ronald M. Laxer ◽  
Paul Thorner ◽  
Marvin J. Fritzler ◽  
Earl D. Silverman

2016 ◽  
Vol 53 (3) ◽  
pp. 339-350 ◽  
Author(s):  
Katherine A. Rodby ◽  
Yvonne E. Kaptein ◽  
James Roring ◽  
Ryan J. Jacobs ◽  
Vicky Kang ◽  
...  

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Tiago Nardi Amaral ◽  
João Francisco Marques Neto ◽  
Aline Tamires Lapa ◽  
Fernando Augusto Peres ◽  
Caio Rodrigues Guirau ◽  
...  

Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs.


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