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Localised scleroderma

Athina Dritsoula, Ioannis Papaioannou, Sandra G Guerra, Carmen Fonseca, Javier Martin, Ariane L Herrick, David J Abraham, Christopher P Denton, Markella Ponticos
OBJECTIVE: NKX2-5 is a homeobox transcription factor required for the formation of the heart and vessels during development, with significant postnatal downregulation and reactivation in disease states characterised by vascular remodelling. In this study, we sought to investigate mechanisms that activate NKX2-5 expression in diseased vessels, such as scleroderma associated pulmonary hypertension (SSc-PH), and identify genetic variability that potentially underlies susceptibility to specific vascular complications...
January 17, 2018: Arthritis & Rheumatology
Giorgia Martini, Simona Campus, Bernd Raffeiner, Gianluca Boscarol, Alessandra Meneghel, Francesco Zulian
Pansclerotic morphoea (PM) is a subtype of juvenile localised scleroderma characterised by severe course with generalised full-thickness skin involvement and possible growth and functional impairment. PM treatment comprises a combination of immunosuppressive agents such as corticosteroids, methotrexate, mycophenolate mofetil, PUVA and antithymocyte globulin and biological agents used in off-label. A possible role of IL-6 in the regulation of firoblast differentiation and stimulation of collagen synthesis has been suggested and in patients with systemic sclerosis (SSc) the treatment with tocilizumab (TCZ) was associated to improvement of skin thickness and joint motion...
September 2017: Clinical and Experimental Rheumatology
Fangfan Ye, Gaoyin Kong, Jia Huang
INTRODUCTION: Scleroderma is a progressive fibrotic disorder of connective tissue which can present multiple anesthetic challenges to anesthetists, especially airway management. Awake intubation with fiberoptic bronchoscope is widely accepted and implemented for progressive systematic scleroderma patients. With the development and improvement of intubation devices these years, there is no report addressing other intubation methods for sclerotic patients. CASE DESCRIPTION: A 47 year-old, 42-kg man with 1-year history of localized scleroderma was scheduled for the operation of inner fixation after 6 days of his acetabular fracture...
2016: SpringerPlus
Julio López-Bastida, Renata Linertová, Juan Oliva-Moreno, Pedro Serrano-Aguilar, Manuel Posada-de-la-Paz, Panos Kanavos, Domenica Taruscio, Arrigo Schieppati, Georgi Iskrov, Márta Péntek, Claudia Delgado, Johann Mathias von der Schulenburg, Ulf Persson, Karine Chevreul, Giovanni Fattore
OBJECTIVE: The aim of this study was to determine the economic burden from a societal perspective and the health-related quality of life (HRQOL) of patients with systemic sclerosis (SSc; scleroderma) in Europe. METHODS: We conducted a cross-sectional study of patients with SSc (involving both localised and systemic sclerosis) from Germany, Italy, Spain, France, the UK, Hungary and Sweden. Data on demographic characteristics, healthcare resource utilisation, informal care, labour productivity losses and HRQOL were collected from the questionnaires completed by patients or their caregivers...
April 2016: European Journal of Health Economics: HEPAC: Health Economics in Prevention and Care
Alexis Strickler, Silvanna Gallo, Pedro Jaramillo, Gonzalo de Toro
INTRODUCTION: Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues. OBJECTIVE: To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment. CLINICAL CASE: The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling...
July 2016: Revista Chilena de Pediatría
Grzegorz Pracoń, Mateusz Płaza, Marta Walentowska-Janowicz, Iwona Sudoł-Szopińska
Systemic sclerosis, popularly referred to as scleroderma, is a chronic connective tissue disease with present autoantibodies against platelet-derived growth factor receptor. These antibodies activate directly fibroblasts causing the dermis and internal organs' fibrosis and vascular damage. Additionally, calcific collections, including hydroxyapatite crystals, may develop in subcutaneous tissue and juxta-articular soft tissue. Herein, we report a case of a 72-year-old woman, referred by a rheumatologist for plain radiography and ultrasound examination of hands due to pain and swelling of the fourth finger of the left hand...
September 2015: Journal of Ultrasonography
Anna Sällfors Holmqvist, Jørgen H Olsen, Lene Mellemkjaer, Stanislaw Garwicz, Lars Hjorth, Christian Moëll, Bengt Månsson, Laufey Tryggvadottir, Henrik Hasle, Jeanette Falck Winther
OBJECTIVES: The pattern of autoimmune diseases in childhood cancer survivors has not been investigated previously. We estimated the risk for an autoimmune disease after childhood cancer in a large, population-based setting with outcome measures from comprehensive, nationwide health registries. METHODS: From the national cancer registries of Denmark, Iceland and Sweden, we identified 20 361 1-year survivors of cancer diagnosed before the age of 20 between the start of cancer registration in the 1940s and 1950s through 2008; 125 794 comparison subjects, matched by age, gender and country, were selected from national population registers...
September 2016: Annals of the Rheumatic Diseases
V Durčanská, H Jedličková, O Sláma, L Velecký, E Březinová, V Vašků
We present a case of a young 26-year-old woman, who has been suffering from localised scleroderma (morphea) for 15 years. Recently, a lesion on the dorsum of her right foot ulcerated. Based on a CT scan and X-ray a diagnosis of ulcerative osteomyellitis was established. The patient was treated with a combination of antibiotics. Subsequent histological examinations showed granulomatous tissue and chronic inflammatory changes on top of pseudoepiteliomatous hyperplasia. The patients status was deteriorating, which resulted in a limb amputation under the knee...
2014: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
M Hørberg, S R Lauesen, J Daugaard-Jensen, I Kjær
BACKGROUND: Linear scleroderma en coup de sabre (SCS) is a rare skin condition, where dense collagen is deposited in a localised groove of the head and neck area resembling the stroke of a sabre. The SCS may involve the oral cavity, but the severity and relation to this skin abnormality is unknown. A paediatric dentist may be the first medical person to identify SCS by its involvement in dentition. It is assumed that the malformation of a dentition could be associated with the severity of the skin deviation...
April 2015: European Archives of Paediatric Dentistry: Official Journal of the European Academy of Paediatric Dentistry
Inderjote Grewal, Omar Khan, William Davis
Pansclerotic morphea is a rare subtype of localised scleroderma. Very few cases have been reported in the literature. This case discusses a young man with a 15-year history of pansclerotic morphea, illustrating the clinical course and complications of this disease. Notably, recurrent squamous cell carcinomas with several high-risk features and frequent cellulitis were the basis of many admissions. As often seen with this disease, autoimmune markers were negative. However, marked eosinophilia was noted late in the course...
2014: BMJ Case Reports
Muhammad Farrukh Nisar, Kimberly Suzanne George Parsons, Chun Xiang Bian, Julia Li Zhong
Long wave UVA radiation (340-400 nm) causes detrimental as well as beneficial effects on human skin. Studies of human skin fibroblasts irradiated with UVA demonstrate increased expression of both antifibrotic heme oxygenase-1 (HO-1) and matrix metalloproteinase 1 (MMP-1). The use of UVA-induced MMP-1 is well-studied in treating skin fibrotic conditions such as localized scleroderma, now called morphea. However, the role that UVA-induced HO-1 plays in phototherapy of morphea has not been characterized. In the present manuscript, we have illustrated and reviewed the biological function of HO-1 and the use of UVA1 wavebands (340-400 nm) for phototherapy; the potential use of HO-1 induction in UVA therapy of morphea is also discussed...
January 2015: Photochemistry and Photobiology
Gianluca Moroncini, Nicolò Schicchi, Giovanni Pomponio, Magdalena Dziadzio, Osmy Paci della Costanza, Alessandra Pierfederici, Lorenzo Ferretti, Giuseppe Pupita, Gianluca Valeri, Giacomo Agliata, Luca Salvolini, Andrea Giovagnoni, Armando Gabrielli
PURPOSE: The authors investigated whether contrast-enhanced cardiovascular magnetic resonance (CMR) imaging may be used to detect early cardiac involvement in patients with systemic sclerosis (SSc). MATERIALS AND METHODS: Twenty-six SSc patients (nine with diffuse cutaneous SSc and 17 with limited cutaneous SSc) and 13 sex- and age-matched healthy controls (HC) were studied. Contrast-enhanced CMR allowed the analysis of first-pass images (areas of hypo-enhancement indicating perfusion defects) and delayed images (persistent hyper-enhancement indicating fibrosis)...
December 2014: La Radiologia Medica
Loredana Elena Stoica, Claudia Valentina Georgescu, V Pătraşcu, C C Radu, I Tolea, L Mogoantă
Basal cell carcinoma (BCC) is the most frequent cutaneous tumour. Having as aim the identification of the clinical-evolutional and histopathologic aspects of the basal cell carcinomas, we have undertaken a retrospective study for a period of 5 years, from 1st January 2004 to 31st December 2008, on 706 patients interned in the Dermatology Clinic of Craiova, whom indicated 738 tumours. A clinical data was drawn for the patients, containing the identification data, environment, profession, cancer localisation and history of the disease, clinical and histopathologic diagnosis...
October 2009: Current Health Sciences Journal
Nicoletta Del Papa, Fabio Caviggioli, Domenico Sambataro, Eleonora Zaccara, Valeriano Vinci, Gabriele Di Luca, Antonina Parafioriti, Elisabetta Armiraglio, Wanda Maglione, Riccardo Polosa, Francesco Klinger, Marco Klinger
Background: Autologous fat tissue grafting (AFTG) has been successfully used in the treatment of different sclerotic conditions, including localised scleroderma. Patients with advanced systemic sclerosis (SSc)-related perioral thickening and mouth opening limitation are candidates to this therapeutic approach. Aims: AFTG of the lips was performed to improve mouth opening in patients with SSc. Materials and Methods: We enrolled in the study 20 patients with diffuse SSc, (median age 35 ±15 years and 11±10 years of disease duration)...
October 22, 2013: Cell Transplantation
Steven Tien Guan Thng, Keryi Wong
Morphoea, or localised scleroderma, is a disease entity with poorly understood pathogenesis. Early diagnosis of the condition is crucial in order to prevent permanent morbidity. However, initial presentations of morphoea can be nonspecific and easily mistaken for other conditions, resulting in late treatment and permanent disability. We report a case of linear morphoea in a 22-year-old man who was initially diagnosed with reflex sympathetic dystrophy. By the time the diagnosis of morphoea was confirmed, the patient had already developed contractures...
March 2013: Singapore Medical Journal
Rahime Inci, Mehmet Fatih Inci, Fuat Ozkan, Perihan Oztürk
Linear scleroderma is a rare variant of localised scleroderma, which is usually seen in childhood and during the adolescent period, and can cause severe functional morbidity as well as cosmetic and psychological problems. Although its ethiopathogenesis is yet obscure, autoimmunity, local ischaemia and injuries, vaccination, irradiation, vitamin K injections, Borrelia burgdorferi and Varicella infections have been incriminated. A 4-year-old girl who had been followed up for about 18 months with diagnosis of epilepsy had a colour discolouration and depression that first appeared 1 year ago and then progressed on her left frontal region...
2012: BMJ Case Reports
Leelavathy Budamakuntla, Dipali Malvankar
The term deep morphea describes a variant of morphea (localised scleroderma) in which inflammation and sclerosis are found in the deep dermis, panniculus, fascia or superficial muscle. It is sometimes associated with autoantibodies. We report the case of a 49 year-old male who had morphea profunda (radiologically and histopathologically confirmed) affecting mainly the left side of the body with face, trunk and limb involvement, along with autoantibody production and associated neurofibromas and lipomas.
September 2012: Indian Dermatology Online Journal
Mary Sommerlad, Richard Bull, Claire Gorman
In this case, we describe an unusual presentation of a young woman with a rash typical of morphoea (confirmed on biopsy), who went on to develop myositis in an atypical distribution. Although the association of myositis with diffuse systemic sclerosis is well described, the link with localised scleroderma (morphoea) and myositis has not been described.
2011: Case Reports in Rheumatology
G McKenna, M Hayes, F M Burke
Scleroderma is a connective tissue disorder that can present with orofacial involvement. A 48 year-old patient presented to Cork University Dental Hospital with concerns about the appearance of her upper central incisor teeth, which had become progressively mobile in recent years. A diagnosis of localised scleroderma had been made a number of years previously by her medical practitioner and the patient reported that her scleroderma-associated microstomia had progressed significantly in recent years. Most reports of this condition advocate the use of sectional impression trays and sectional dentures to replace missing teeth...
June 2012: European Journal of Prosthodontics and Restorative Dentistry
Sabina Trainito, Lorenzo Favero, Giorgia Martini, Thomas Klit Pedersen, Vittorio Favero, Troels Herlin, Francesco Zulian
AIM: Localised scleroderma of the face (LSF) can lead to significant aesthetical and functional abnormalities. Despite their quite frequent clinical observation, the odontostomatologic complications are not thoroughly described. Aim of the study was to describe the clinical features of the most frequent odontostomatologic abnormalities of LSF and to propose clinical and radiologic criteria for the assessment and follow-up of these complications. METHODS:   We performed a cross-sectional, multicenter study involving a multidisciplinary team formed by paediatric rheumatologists, orthodontists and radiologists...
July 2012: Journal of Paediatrics and Child Health
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