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Srisuttiyakorn, Chutika, and Kobkul Aunhachoke. "Scleroderma with Nodular Scleroderma." Case Reports in Dermatology 8, no. 3 (2016): 303–10. http://dx.doi.org/10.1159/000452324.
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Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. Main Observations: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the scleroderma condition. Although no additional treatment was given, the lesions remained stable without progression. Conclusions: Although this condition is rare, it has been reported sporadically, and clinicians should be able to recognize this variant in cases of scleroderma presenting with firm nodules or plaques.
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Kassira, Sama, Tarannum Jaleel, Peter Pavlidakey, and Naveed Sami. "Keloidal Scleroderma: Case Report and Review." Case Reports in Dermatological Medicine 2015 (2015): 1–4. http://dx.doi.org/10.1155/2015/635481.
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Objective. We report a rare case of keloidal scleroderma and provide an analysis of similar cases.Results. A 41 year-old woman presented with dark brown, indurated, exophytic nodules over the chest along with smaller hyperpigmented plaques scattered over the abdomen, with concomitant sclerodactyly. The clinical, laboratory, and pathological findings were consistent with a diagnosis of keloidal scleroderma. The patient was treated with methotrexate, resulting in reduced firmness of her plaques and no new lesions. A literature review of previously reported cases was performed using keywords including keloidal morphea, keloidal scleroderma, nodular morphea, and nodular scleroderma. In our review, the majority of patients were African American and female. 91% of cases had nodular lesions with distribution on the trunk. The majority of patients exhibited sclerodactyly and pulmonary involvement was reported in 28%1. The majority of patients were ANA positive (63%) and only 10% demonstrated anti-SCL-70 positivity.Conclusion. Keloidal scleroderma is a rare presentation, which can often be clinically confused with keloid and scar formation. Due to this being a rare variant, our knowledge of treatment options and efficacy is limited. Methotrexate could be considered as an initial treatment option for patients with progressive keloidal scleroderma.
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Rambhia, KinjalD, AtulM Dongre, and UdayS Khopkar. "Sclerodermoid plaques: A riddle of ′H′." Indian Journal of Dermatology, Venereology, and Leprology 81, no. 3 (2015): 327. http://dx.doi.org/10.4103/0378-6323.152748.
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Francoeur, Cleve J. "Sclerodermoid Plaques in a Middle-aged Man." Archives of Dermatology 127, no. 10 (1991): 1573. http://dx.doi.org/10.1001/archderm.1991.01680090137020.
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Skakodub, A. A., N. А. Geppe, O. I. Admakin, et al. "Clinical and X-ray diagnostic criteria for maxillofacial damage in children with juvenile limited scleroderma." Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) 65, no. 2 (2020): 71–79. http://dx.doi.org/10.21508/1027-4065-2020-65-2-71-79.
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The objective of our study was to improve the diagnosis of maxillofacial lesions in children with juvenile scleroderma. We performed a dental examination of 41 children from 4 to 17 years old with juvenile scleroderma. Based on the clinical X-ray examination we identified the main diagnostic signs of the maxillofacial damage in children with juvenile scleroderma, including partial hemiatrophy, plaque or linear facial lesions, reduced salivation, atrophic glossitis, plaque spots of mucous tongue atrophy, ischemia or shortening of the sublingual bridle, local recession of the gums of the lower jaw, dystopia and tooth supraposition, disocclusion, delay teething, spontaneous resorption of the permanent teeth roots, one-sided delay in the development of jaw bones. Using this complex of symptoms a dentist at the first visit can pre-diagnose scleroderma, which is especially important for the selection of adequate methods of treatment and prevention.
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Gupta, Vishal, Suman Patra, Mohammad Firdaus Ali, and Gomathy Sethuraman. "Sclerodermoid Hypertrichotic Plaques with Insulin-Dependent Diabetes Mellitus." Pediatric Dermatology 32, no. 5 (2015): 731–32. http://dx.doi.org/10.1111/pde.12574.
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Valančienė, Greta, Daiva Jasaitienė, and Skaidra Valiukevičienė. "Pathogenesis and treatment modalities of localized scleroderma." Medicina 46, no. 10 (2010): 649. http://dx.doi.org/10.3390/medicina46100092.
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Localized scleroderma is a chronic inflammatory disease primarily of the dermis and subcutaneous fat that ultimately leads to a scar-like sclerosis of connective tissue. The disorder manifests as various plaques of different shape and size with signs of skin inflammation, sclerosis, and atrophy. This is a relatively rare inflammatory disease characterized by a chronic course, unknown etiology, and insufficiently clear pathogenesis. Many factors may influence its appearance: trauma, genetic factors, disorders of the immune system or hormone metabolism, viral infections, toxic substances or pharmaceutical agents, neurogenic factors, and Borrelia burgdorferi infection. Various therapeutic modalities are being used for the treatment of localized scleroderma. There is no precise treatment scheme for this disease. A majority of patients can be successfully treated with topical pharmaceutical agents and phototherapy, but some of them with progressive, disseminated, and causing disability localized scleroderma are in need of systemic treatment. The aim of this article is not only to dispute about the clinical and morphological characteristics of localized scleroderma, but also to present the newest generalized data about the possible origin, pathogenesis, and treatment modalities of this disease.
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AB, Guerra. "Congenital Morphea: Two Case Reports." Neonatology and Clinical Pediatrics 7, no. 3 (2020): 1–3. http://dx.doi.org/10.24966/ncp-878x/100062.
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Congenital morphea is a rare and underestimated form of localized scleroderma that presents at birth. From the five known subtypes, linear and plaque morphea are the most common and benign forms and are both described in these cases. Extracutaneous manifestations are rare.
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AB, Guerra. "Congenital Morphea: Two Case Reports." Neonatology and Clinical Pediatrics 7, no. 3 (2020): 1–3. http://dx.doi.org/10.24966/ncp-878x/100062.
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Congenital morphea is a rare and underestimated form of localized scleroderma that presents at birth. From the five known subtypes, linear and plaque morphea are the most common and benign forms and are both described in these cases. Extracutaneous manifestations are rare.
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Rencic, Adrienne, Nooshin Ketabchi Brinster, and Carlos H. Nousari. "Keloid Morphea and Nodular Scleroderma: Two Distinct Clinical Variants of Scleroderma?" Journal of Cutaneous Medicine and Surgery 7, no. 1 (2003): 20–24. http://dx.doi.org/10.1177/120347540300700104.
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Background: For nearly a century, the terms “keloid morphea” and “nodular scleroderma” have been used interchangeably without defined clinical or histologic criteria. Objective: To define the conditions “keloid morphea” and “nodular scleroderma” by correlating the clinical and histologic features. Methods: We retrospectively identified six patients with keloidal lesions and nodules from 70 consecutive patients with scleroderma seen in the dermatology clinic. The clinical presentation and histopathological findings were reviewed. Results: Six of 70 patients with scleroderma (45 systemic and 25 morphea) exhibited keloidal or nodular lesions. All these patients had systemic sclerosis. Clinically one patient (case 1) had nodules; five (cases 2–6) had keloids. The nodular lesions had histologic findings consistent with keloid, while the keloidal plaques were variably keloids or morphea histopathologically. There was no correlation between the clinical morphology and the histologic findings, except for cases 5 and 6. These patients were African–American, with a family history of keloids and typical keloids clinically and histologically that developed from sites with normal skin. Conclusion: Keloid morphea and nodular scleroderma are clinical terms that describe keloidal and nodular lesions in patients most commonly with scleroderma. The clinicopathologic association is variable. Based on a review of the English literature and our series of six patients, we identified two clinical variants; (1) keloidal or nodular lesions arising from sclerodermatous skin with histologic findings of keloid or scleroderma, (2) typical keloids clinically and histologically, arising in normal skin in patients with a family history of keloids. Awareness of these entities is important for proper diagnosis of the cutaneous lesions and for recognizing that the cutaneous findings may be a sign of systemic sclerosis.
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Milenkovic, S., L. Petrovic, D. Risimic, et al. "Choroidal Sclerosis in Localized Scleroderma (Morphea en Plaque)." Ophthalmic Research 40, no. 2 (2008): 101–4. http://dx.doi.org/10.1159/000113889.
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Gangadharan Nair, H., M. K. Rai, M. Singh, et al. "SAT0319 SUBCLINICAL ATHEROSCLEROSIS IN INDIAN PATIENTS WITH SCLERODERMA – CLINICAL AND SEROLOGICAL ASSOCIATIONS." Annals of the Rheumatic Diseases 79, Suppl 1 (2020): 1105.2–1105. http://dx.doi.org/10.1136/annrheumdis-2020-eular.296.
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Background:Scleroderma has been associated with increased risk of cardiovascular events, however,studies on this from India are sparse.We evaluated clinical and serological factors associated with subclinical atherosclerosis in Indian patients with scleroderma, in a cross-sectional design.Objectives:To compare carotid intima-medial thickness (CIMT, mean value of both carotids) as a measure of subclinical atherosclerosis (SCA) between patients with scleroderma (n=61) fulfilling 2013 ACR/EULAR criteria, and healthy controls (n=41).- To compare clinical (body mass index – BMI, waist-hip ratio – WHR, fasting lipid profile) and serological factors (microparticles, endothelial microparticles, inflammatory cytokines associated with increased cardiovascular risk) between patients with scleroderma and healthy controls.- To identify factors associated with SCA in scleroderma patients.Methods:Subclinical atherosclerosis(SCA) was defined by presence of carotid plaques, or increased CIMT >2 standard deviations compared with Indian reference standards for age and sex. Total microparticles (TMP) were measured of plasma after ultracentrifugation as per previously described protocol using microbeads of 3 μm size (TMP were of size 0.1-1 μm); of these, microparticles positive for CD31 and CD142 were endothelial microparticles (EMP). Serum cytokines (IL-1, IL-6, TNF-α, IL-17) were measured by ELISA using manufacturer instructions. Linear regression was used to identify the determinants of CIMT in scleroderma. Binomial logistic regression was used to identify factors associated with subclinical athersclerosis in scleroderma.VariablePatients with scleroderma (n=61)Healthy controls (n=41)p valueAge37.8 ± 11.9235.37 ± 6.690.2375Gender (M:F)11:506:350.6516Diabetes/Hypertension/Tobacco use1/2/00/0/0NSBody mass index (kg/m2)20.11 ± 3.8224.38 ± 4.45<0.0001Waist-hip ratio0.86 ± 0.110.89 ± 0.070.1251Total cholesterol (mg/dL)142.5 ± 30.7147.3 ± 39.50.4948Triglycerides (mg/dL)99.4 ± 37121.4 ± 460.0087HDL cholesterol (mg/dL)46.9 ± 4.946.1 ± 4.20.4029LDL cholesterol (mg/dL)93.6 ± 10.593.3 ± 7.50.8520VLDL cholesterol (mg/dL)19.9 ± 7.424.7 ± 9.70.0057Carotid intima-medial thickness (mm)0.68 ± 0.100.53 ± 0.03<0.0001Total microparticles (per±L)12913 ± 24936272 ± 1533<0.0001Endothelial microparticles (per±L)2623 ± 1032829 ± 439.5<0.0001Serum IL-1±(pg/mL)38.19 ± 13.4631.38 ± 18.290.0326IL-6 (pg/mL)176.6 ± 85.74128.9 ± 53.610.0020IL-17 (pg/mL)56.3 ± 20.4553.89 ± 20.510.5611TNF±(pg/mL)49.65 ± 26.7142.09 ± 30.410.1879Results:Despite lower BMI, triglycerides and VLDL cholesterol, CIMT was significantly higher in patients with scleroderma. Patients with scleroderma had significantly higher total microparticles and endothelial microparticles in plasma, and serum IL-1± and IL-6 (Table 1). On multivariable regression, age was the only significant determinant of CIMT. 28 (45.9%) patients had SCA; 13 (21.3%) had carotid plaques. Patients with SCA had higher proportion of males (9/28 in those with SCA vs 2/33 in those without SCA). Binomial logistic regression did not identify any other significant predictors of SCA.Table 1Comparison between patients with scleroderma and healthy controlsSerum IL-1± (pg/mL)38.19 ± 13.4631.38 ± 18.290.0326IL-6 (pg/mL)176.6 ± 85.74128.9 ± 53.610.0020IL-17 (pg/mL)56.3 ± 20.4553.89 ± 20.510.5611TNF± (pg/mL)49.65 ± 26.7142.09 ± 30.410.1879Conclusion:Patients with scleroderma had significant burden of subclinical atherosclerosis, which could not be explained by traditional or novel cardiovascular risk factors.References:[1]Psarras A, Soulaidopoulos S, Garyfallos A, Kitas G, Dimitroulas T. A critical view on cardiovascular risk in systemic sclerosis. Rheumatol Int. 2017 Jan; 37(1):85-95.[2]Jung C, Drummer K, Oelzner P, Figulla HR, Boettcher J, Franz M, et al. The association between endothelial microparticles and inflammation in patients with systemic sclerosis and Raynaud’s phenomenon as detected by functional imaging. Clin HemorheolMicrocirc. 2015; 61(3):549-557.Acknowledgments:Supported by IRA(Indian Rheumatology Association) Research Grant to DP Misra.Disclosure of Interests:None declared
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Krakhaleva, Yulia A., Anastasia V. Kolerova, Elena D. Sorokina, et al. "Possibilities of skin ultrasound examination in the management of patients with localized scleroderma." Russian Journal of Skin and Venereal Diseases 24, no. 1 (2021): 17–24. http://dx.doi.org/10.17816/dv57058.
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With the help of ultrasound examination (ultrasound), it is possible to visualize the skin of patients with suspected localized scleroderma, as well as to objectively determine the stage of the disease and assess the effectiveness of therapy.
 Three patients with linear, plaque forms of scleroderma, as well as lichen sclerosus, underwent skin ultrasound using a Skinscanner DUB TPM device with a 75 MHz transducer, 4 mm penetration, 21 m resolution in the area of lesions and in adjacent areas of healthy skin, using their ratio coefficient (RC) for comparative evaluation. The thickness of the epidermis and dermis, their echogenicity were determined.
 With a linear form of scleroderma, an increase in the thickness of the epidermis in the area of the focus (RC 0.850.0125) and a decrease in its echogenicity (RC 1.580.46) were observed. The echogenicity of the dermis was significantly reduced in the lesion (RC 3.021.17). The dermis thickness was slightly less in the center of the lesion (RC 1.09), at the periphery of the lesion it was moderately increased (RC 0.86). In the plaque form of scleroderma, a decrease in the echogenicity of the epidermis was observed in the foci (RC 1.320.49); an increase in the thickness of the dermis (RC 0.790.16) and a decrease in its e echogenicity (RC 1.260.57). In 7 of 11 foci, a subepidermal hypoechoic band was visualized. With lichen sclerosis in the foci, an increase in the thickness of the epidermis (RC 0.420.12) and its acoustic density (RC 0.630.0793), a decrease in the thickness and echo density of the dermis (RC 1.320.00943 and RC 1.550.6, respectively).
 With different forms of LS, a different ultrasound picture was observed, depending on the stage and activity of the process. The changes identified during treatment reflect the effectiveness of the therapy and the rate of restoration of the skin structure during therapy.
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Aouali, Soraya, Saida Sefraoui, Kaoutar Sof, Siham Dikhaye, and Nada Zizi. "Sclerosis and blisters: An uncommon association revealing a new case of bullous morphea." Our Dermatology Online 12, no. 2 (2021): 167–68. http://dx.doi.org/10.7241/ourd.20212.15.
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Bullous morphea is a rare variant of localized scleroderma (morphea) characterized by subepidermal bullae developed over sclerotic plaques. The pathogenesis of bullae formation has been the subject of several debates, which arrived at the conclusion of a multifactorial mechanism. We report the case of a 67-year-old patient with bullous morphea of the trunk and thighs, who showed a good response to PUVA therapy combined with topical steroids. Our case report supports the efficiency of PUVA therapy associated with topical steroids as a safe regimen compared to other therapeutic approaches.
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El-Darouti, Mohammad A. "Hyperpigmented, hypertrichotic, and sclerodermoid plaques: An unusual variant of Muckle–Wells syndrome." Journal of the American Academy of Dermatology 61, no. 4 (2009): 725–27. http://dx.doi.org/10.1016/j.jaad.2009.03.020.
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Obadeh Mahmoud, Al-Omary, and S. A. Bondar. "Proliferative function of endothelium in local scleroderma, gender and age characteristics of the disease." Ukrainian Journal of Dermatology, Venerology, Cosmetology, no. 2 (June 17, 2021): 25–33. http://dx.doi.org/10.30978/ujdvk2021-2-25.
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Objective — to investigate the variability of vascular endothelial growth factor (VEGF) in localized scleroderma (LS), characteristics of the disease in different age groups. Materials and methods. The study included the clinical evaluation of 78 patients (27 men, 51 women) with localized scleroderma aged (43.2 ± 7.28) years. All patients underwent clinical, laboratory, and enzyme immunoassay investigation of VEGF. The criteria for inclusion in the study were: the presence of localized scleroderma and the absence of signs of a systemic process. Results and discussion. It has been proven that localized scleroderma is genderrelated, more often recorded in women (65.4 %, p < 0.05), with an attributive risk of 50.0 % compared to men. Late diagnosis of the disease was proven in 78.2 % of patients. The age and gender characteristics of this pathology were determined — men are more likely to get sick at a young age (up to 20 years; 22.2 % of the surveyed), however, women over the age of 55 prevail (p < 0.05). Women sought dermatological help earlier compared to men (29.4 %; p < 0.05), but had a higher risk of early progression (p < 0.05). Among the clinical forms of localized scleroderma, most of the cases (70.5 %) were of the plaque form. In 15.4% of cases, a linear form was diagnosed, in 9.0% lichen sclerosus, in 5.1 % — Pasini—Pierini idiopathic atrophoderma. The plaque form of LS was significantly more often detected in women (80.3 %), and the linear form and lichen sclerosus — in men (22.2 % each). The average number of lesions was 2.8 ± 1.12. Three lesions were found in almost half (43.6 %) of the patients, one lesion – in 23.1%, two lesions – in 16.7%, four lesions – in 11.5%. Five or more lesions were present in 4 (5.1 %) patients. It has been found that the highest content of VEGF was observed in classic plaque form (p < 0.05), the lowest — in Pasini—Pierini idiopathic atrophoderma (p < 0.05). Ushaped age dependence of the disease was determined: high content of VEGFA in patients under the age of 20 (p < 0.05), with a significant decrease of VEGFA in patients aged 20—35 years (p < 0.05) and further increase in those aged 35 to 55 years (p < 0.05). It was established that VEGFA level increased at patients with early progression of the disease — in the first 2 years after the start of clinical manifestations against the group of patients with slow progression — more than 6 years (p < 0.05). In assessment of gender dependence, we found a higher content of VEGFA in female patients compared to male patients (p < 0.05). Conclusions. Localized scleroderma has gender and agerelated characteristics and is 50.0 % more often recorded in women. Among men, young patients predominate. Studying the pathogenesis of LS and assessing the proliferative function of the endothelium of the disease will make it possible to predict the course of the disease, improve the diagnosis and treatment.
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SHIRASAKI, Fumiaki, Yuji YAMAGISHI, and Norio OHTSUKI. "A Case of Nodular Scleroderma with Large Annular Plaques on the Trunk." Nishi Nihon Hifuka 58, no. 4 (1996): 593–97. http://dx.doi.org/10.2336/nishinihonhifu.58.593.
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Liliana, Mocanu, Deacu Sorin, Roman Polimaru, and Aschie Mariana. "A Polarized Light Microscopy Study in a Case of Morphea." ARS Medica Tomitana 26, no. 1 (2020): 1–4. http://dx.doi.org/10.2478/arsm-2020-0001.
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Abstract We report a case of plaque type of scleroderma with specific clinical features and conventional histopathology, with sclerosis and hipocellularity of fibroblasts and preservation of elastic tissue. We describe polarized light microscopy findings, on conventional stained slides and on picro sirius red stained slides. We appreciate that picro sirius red stain allows a better characterization of collagen fibres composition in papillary and reticular dermis, that is severely disturbed in morphea, with an inverse distribution of collagen fibres type I and III comparative with normal dermis.
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Tabata, Nobuko, and Chiyoko Nagano Inoue. "Juvenile Localized Scleroderma with Hyaline Deposits in the Renal Arteriole." Case Reports in Dermatology 10, no. 1 (2018): 89–95. http://dx.doi.org/10.1159/000488901.
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We report a 10-year-old boy with localized scleroderma of the linear and plaque type, who showed proteinuria and hematuria. In this patient, skin, articular, and renal manifestations appeared successively and then began to resolve in the same order. A renal biopsy specimen demonstrated mild mesangial cell proliferation, exudate of immunoglobulin in the glomerular capillary, and large electron-dense deposits in the afferent arteriole. We consider that there were some transient factors that had caused the skin and articular manifestations, which also induced renal vascular inflammatory responses.
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Zaslavsky, Denis V., A. A. Sidikov, L. V. Garyutkina, A. I. Sadykov, I. N. Chuprov, and D. V. Kozlova. "New aspects of localized scleroderma pathogenesis: practical basis." Russian Journal of Skin and Venereal Diseases 23, no. 4 (2020): 227–37. http://dx.doi.org/10.17816/dv48907.
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BACKGROUND:Recently there has been an increase in the number of patients with scleroderma.
 AIM:This study aimed to investigate the pathogenesis and course and present the immunopathogenesis of localized scleroderma (LS) or morphea.
 MATERIALS AND METHODS:From 2010 to 2019, a prospective study of 77 patients with LS was conducted on the basis of the Leningrad regional center for specialized types of medical care. Based on histological examination, LS diagnosis was verified in 40 of 77 patients. Patients with LS (n = 40) were included in the first research group and were then divided into two subgroups based on the limitation period for the first symptoms of the disease: in subgroup I (n = 20), the disease manifested no later than 1.7 months before clinic visit; in subgroup II (n = 20), the limitation period was 1.5 years.
 RESULTS:Patients of both subgroups (n = 40) underwent immunohistochemical (IL-2, IL-4, CD4, CD8, vimentin, Toll-like receptor TLR7) tissue analysis and immunological blood tests to determine autoantibodies. To improve the differential diagnosis of LS, a comparative assessment of clinical manifestations and histological signs was performed in patients with LS (n = 40) and patients with clinically similar dermatoses (n = 37): annular granuloma (n = 12, 7 women and 5 men, average age 44 12 years), small plaque (n = 15, 6 women and 9 men, average age 42 4 years), and large plaque (n = 10, 5 women and 5 men, average age 59 8 years) parapsoriasis. According to the results of the histological examination, inflammatory changes are dominant in patients with LS manifestation period of 1.7 months from the onset of the disease, while fibrotic changes are apparent in patients with a manifestation period of 1.5 years. The expressions of CD4, CD8, IL-2, and TLR7 were more pronounced in subgroup 1, while those of IL-4, CD4, and vimentin were high in subgroup 2. No autoantibodies were detected in the blood of patients with LS. The results allow us to divide the pathogenesis of LS into two phases: inflammatory and fibrotic. Immune dysregulation and fibrosis occur simultaneously, but with phase dependant predominance.
 CONCLUSIONS:In the future, a detailed understanding of the pathogenesis of LS will help improve diagnostic and therapeutic algorithms and reduce the frequency of relapse and complications.
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Smržová, A., M. Schubertová, J. Vymětal, et al. "AB0585 Intima Media Thickness and Atherosclerotic Plaques Are Associated with Subclinical Atherosclerosis and Depression in Systemic Scleroderma." Annals of the Rheumatic Diseases 75, Suppl 2 (2016): 1104.3–1105. http://dx.doi.org/10.1136/annrheumdis-2016-eular.5292.
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Chiriac, Anca, Piotr Brzezinski, Anca E. Chiriac, et al. "The Value of Ultrasonography in the Diagnosis and Monitoring of Localized Morphea — Case Report." Journal of Interdisciplinary Medicine 1, no. 2 (2016): 193–96. http://dx.doi.org/10.1515/jim-2016-0037.
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AbstractIntroduction:The aim of this presentation is to highlight the usefulness of high-frequency ultrasound (18 MHz) in localized morphea for: identification of the lesion, guided skin biopsy, quantification of skin thickness, evaluating the severity by measuring total echogenicity.Case presentation:A 62-year-old Caucasian woman was referred to the Dermatology Department for a well-circumscribed indurate plaque localized on the right side of the abdominal wall and thigh. On clinical examination, a large well-delimited, indurate plaque, silvery in the center and surrounded by a purplish-red halo (lilac ring) was noticed on the right side of the abdomen and thigh. An ultrasound-guided punch biopsy was carried out and the microscopic examination of the biopsy revealed moderate interstitial inflammatory infiltrate together with abundant collagen bundles in the dermis and subcutis and a diagnosis of localized morphea (scleroderma) was established. Ultrasonography was performed and skin thickness was measured using high-frequency US (18 MHz) and was found to be 3.1 mm to 3.9 mm.Conclusion:high frequency ultrasound is an inexpensive, easy to perform, noninvasive method, replacing surgical biopsy and offering a valuable quantification of skin fibrosis.
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Braun, Alicia, James Poulton, and C. Lisa Kauffman. "Idiopathic Atrophoderma of Pasini and Pierini." Journal of Cutaneous Medicine and Surgery 2, no. 2 (1997): 104–7. http://dx.doi.org/10.1177/120347549700200211.
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Background: Idiopathic atrophoderma of Pasini and Pierini (LAPP) is a rare pattern of dermal atrophy, with less than 300 cases reported worldwide. Currently, there is controversy surrounding the classification of LAPP; some consider it to be a variant of morphea, while others view it as a separate disease entity. Objective: Our purpose was to further elucidate the nature and course of this unusual disease. Methods: We present a case report, with long-term follow-up of a case of IAPP of 30 years' duration. Results: The patient's lesions were primarily atrophic, without progression to morphea, scleroderma, or other systemic disease. Biopsy of a long-standing plaque demonstrated dermal thinning, and minimal dermal infiltrate. Conclusion: This case supports the findings of the literature on this subject: the majority of patients with IAPP have an entirely benign course, without progression to other disease.
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Unterberger, I. "Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids." Journal of Neurology, Neurosurgery & Psychiatry 74, no. 5 (2003): 661–64. http://dx.doi.org/10.1136/jnnp.74.5.661.
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Efanova, E. N., E. A. Vasilieva, and M. Y. Rusak. "THE COMBINATION OF LICHEN PLANUS, PLAQUE ETIOLOGY OF LICHEN SCLEROSUS AND SCLERODERMA PATIENT WITH AUTOIMMUNE THYROIDITIS." Journal of scientific articles "Health and Education millennium" 19, no. 7 (2017): 29–32. http://dx.doi.org/10.26787/nydha-2226-7425-2017-19-7-29-32.
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Mohamed, Abdimajid Ahmed, Xin-liang Lu, and Faycal Awaleh Mounmin. "Diagnosis and Treatment of Esophageal Candidiasis: Current Updates." Canadian Journal of Gastroenterology and Hepatology 2019 (October 20, 2019): 1–6. http://dx.doi.org/10.1155/2019/3585136.
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Esophageal candidiasis (EC) is the most common type of infectious esophagitis. In the gastrointestinal tract, the esophagus is the second most susceptible to candida infection, only after the oropharynx. Immunocompromised patients are most at risk, including patients with HIV/AIDS, leukemia, diabetics, and those who are receiving corticosteroids, radiation, and chemotherapy. Another group includes those who used antibiotics frequently and those who have esophageal motility disorder (cardiac achalasia and scleroderma). Patients complained of pain on swallowing, difficulty swallowing, and pain behind the sternum. On physical examination, there is a plaque that often occurs together with oral thrush. Endoscopic examination is the best approach to diagnose this disease by directly observing the white mucosal plaque-like lesions and exudates adherent to the mucosa. These adherent lesions cannot be washed off with water from irrigation. This disease is confirmed histologically by taking the biopsy or brushings of yeast and pseudohyphae invading mucosal cells. The treatment is by systemic antifungal drugs given orally in a defined course. It is important to differentiate esophageal candidiasis from other forms of infectious esophagitis such as cytomegalovirus, herpes simplex virus, gastroesophageal reflux disease, medication-induced esophagitis, radiation-induced esophageal injury, and inflammatory conditions such as eosinophilic esophagitis. Except for a few complications such as necrotizing esophageal candidiasis, fistula, and sepsis, the prognosis of esophageal candidiasis has been good.
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Özdemir, M., B. Engin, H. Toy, and I. Mevlitoglu. "Treatment of plaque-type localized scleroderma with retinoic acid and ultraviolet A plus the photosensitizer psoralen: a case series." Journal of the European Academy of Dermatology and Venereology 22, no. 4 (2008): 519–21. http://dx.doi.org/10.1111/j.1468-3083.2007.02390.x.
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Zanatta, Thais P., Stela M. Kulczynski, Caroline W. Guterres, et al. "Morphological and Patogenic Characterization of Sclerotinia sclerotiorum." Journal of Agricultural Science 11, no. 8 (2019): 302. http://dx.doi.org/10.5539/jas.v11n8p302.
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White mold is a disease with a wide distribution worldwide. Temperatures between 18-23 &deg;C and high humidity conditions favor the occurrence of the pathogen. For the control of the disease it is fundamental to understand the morphology and pathogenicity of the fungus. The objective of this study was to characterize the morphological and pathogenic characteristics of Sclerotinia sclerotiorum isolates from the state of Rio Grande do Sul. Sclerodes were disinfested, placed in the center of plates containing culture medium and incubated under controlled conditions. The evaluations were performed daily, during a period of 30 days, from the incubation of sclerotia. The experimental design was completely randomized, with four plaques per isolate, each plate one replicate. The characteristics evaluated for the mycelium characterization were: time required for the fungus to occupy the plate; density of the formed mycelium; coloration of the colonies and mycelial growth rate. Scleroderma assessments were based on training or not; time for formation of the first sclerodium; total amount formed per plate; Format; distribution in the colony and weight. The isolates were pathogenically characterized by the methodology of inoculation of the detached leaf. All data were submitted to analysis of variance and the means were compared by the Skott-Knott test, at 1% probability. The evaluated populations presented wide variability for the studied characteristics. It was not possible to verify the existence of common groups that could be related to the origin of the isolates, due to the high genetic diversity. The isolates showed different levels of aggressiveness, the two being more aggressive LF02 and LF06.
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Wilkins, Karl, Joel DeKoven, and Dalai Assaad. "Cutaneous Reactions Associated with Vitamin K1." Journal of Cutaneous Medicine and Surgery 4, no. 3 (2000): 163–67. http://dx.doi.org/10.1177/120347540000400311.
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Background: Vitamin K1 (phytonadione) is a fat-soluble, naturally occurring vitamin used to treat certain coagulation disorders. A review of the adverse cutaneous reactions to Vitamin K1 is important because this diagnosis can be easily overlooked. This is due to their low incidence and because the presentation and morphology can vary considerably. Objective: The objective of this article is to summarize the different morphologies, the natural history, and the treatment of the cutaneous reactions reported to Vitamin K1 Methods: A case of a patient who developed a localized eczematous plaque at the site of a vitamin K1 injection is outlined. A review of the English medical literature focused on the adverse cutaneous reactions associated with intramuscular or subcutaneous use of vitamin K1 Results: Our patient developed a localized eczematous reaction to subcutaneous vitamin K1 The eruption developed within 7 days of her dose of vitamin K1 The eruption persisted for 18 months despite treatment with topical and intralesional steroids. There are three distinct types of cutaneous reactions to vitamin K1 localized eczematous, localized morphea-form, and, very rarely, diffuse maculopapular eruption. The eczematous type (32 cases) appears at the site of injection, and the median number of days between injection and appearance of the eruption is 13 days. The dose range required to initiate the reaction is broad (10 to 410 mg). Thirteen of 32 cases took more than 2 months to resolve. The morphea-form type (7 cases) is a localized morphea-form patch that appears at the site of injection. The average delay before presentation of morphea-form changes was 8.5 months (range: 5 weeks-1.5 years). The dose range is broad (30–2080 mg), and the prognosis for resolution very poor. Conclusion: The diagnosis of an adverse cutaneous reaction to vitamin K can be made if the possibility is considered. Many of these reactions are very slow to clear up and some may persist as a chronic sclerodermoid change. Managing these reactions may be frustrating for both the patient and the clinician.
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Nitish, Vimal Raj, David Munoz, Pablo González-López, Nabeel Alshafai, Agdaliya Mikhalkova, and Julian Spears. "Ipsilateral brain cavernoma under scleroderma plaque: a case report." Pan African Medical Journal 32 (2019). http://dx.doi.org/10.11604/pamj.2019.32.13.15288.
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Latha, S. Suzsmi, S. Sivaramakrishnan, T. V. Ramesh, and K. Manoharan. "A CASE OF MORPHOEA EN COUP DE SABRE AND PLAQUE TYPE MORPHOEA." INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH, April 1, 2021, 26–27. http://dx.doi.org/10.36106/ijsr/8626791.
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Morphoea is a connective tissue disease that is uncommon with the most prominent feature being thickening or brosis of the skin without internal organ involvement. It is also known as localised scleroderma. Morphoea is classied into several forms based on their clinical presentation and depth of tissue involvement. Overproduction of altered collagen by broblast is the cause of abnormality in morphea, and the cause for broblast hyperactivity is still unknown, although there are several mechanisms already proposed. We hereby report a case of Morphoea en coup de sabre in association with plaque type morphoea.
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Vinje, Odd, and Berit Flatø. "Epidemiologi ved sklerodermi hos barn." Norsk Epidemiologi 18, no. 1 (2009). http://dx.doi.org/10.5324/nje.v18i1.82.
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<li>Localized scleroderma is an infrequent disease in children, but still ten times more frequent than systemic sclerosis. The incidence is probably 5-10/100,000 per year. The disease is classified into five subgroups. Paediatric rheumatologists report linear scleroderma as the most frequent subgroup whereas plaque morphea is found most frequently by dermatologists, linear scleroderma being in second place. The risk of transforming into systemic sclerosis is minimal. The mortality risk is not found increased compared with the normal population. Systemic sclerosis is the most infrequent systemic connective tissue disease in children, the incidence is less than 1/1,000,000 per year. The outcome in children is considered to be generally better than in adults. However, deaths is caused most frequently by heart failure with or without pulmonal hypertension, but also by renal or respiratory insuffiency, CNS disease and sepsis. Cumulative survival after 5, 10, 15 and 20 years of disease is reported to be 89%, 80%, 74% and 69%. For the age group less than 16 years the median age of death is reported to be 10.4 years, range 5 to 15 years. The disease in the children who die is often rapidly progressing with early involvement of internal organs. </li>
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Jain, Nibha, Shilpa Jagadeesh, and Arumugam Moorthy. "2. Pregnancy and myositis." Rheumatology Advances in Practice 3, Supplement_1 (2019). http://dx.doi.org/10.1093/rap/rkz030.001.
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Abstract Introduction Inflammatory disorders can appear as a spectrum and pose diagnostic challenges. Inflammatory myositis affects women of childbearing age. This situation presents challenges in management of disease during pregnancy. Myositis specific antibodies are expected to lead to certain clinical presentation but cases outside known information always occur as we learn from this case report. Case description A 26-year-old Asian lady was referred to rheumatology for inflammatory arthritis and Raynaud’s on a background of scalp psoriasis and family history of psoriatic arthritis. She had hip pain with no spinal inflammation and was commenced on low dose methotrexate. ANA 1:6400 but autoimmune screen negative. She was intolerant to higher doses of methotrexate. She developed severe hip pain which was not septic and eventually developed reduced hip movements and forward flexion of spine. X-ray showed soft tissue calcification around hip methotrexate was stopped due to respiratory symptoms but HRCT and pulmonary function were normal. Unfortunately, she was lost to follow-up. She was then referred after a year with skin tightening (indurated plaque) over loin and chest. CK was elevated without clinical evidence of muscle weakness and ANA 1:1600 positive with negative ENA and normal DsDNA/Compliments. Dermatologists felt that indurated plaque was morphea as the histology was inconclusive. She developed pelvic girdle weakness along with left hip calcification and progression of skin tightening of fingers and forearm. Although biopsy and MRI thigh were negative for myositis, nerve conduction studies showed severe active polymyositis. Her extended myositis panel now showed Mi-2 antibody. As she was intolerant to azathioprine with a progressive illness and was keen to have children soon, she was started on IvIG. Despite being on IvIg she developed refractory calcium discharging sinus over her hip. Rituximab was not licensed for myositis then and she was not keen to start on any other medications recommended by the myositis specialist centre. She had a successful pregnancy after 6 months of disease control under joint care of maternal foetal medicine and rheumatology. On repeat autoimmune testing prior to pregnancy, anti-Ro was equivocal (previously negative) hence antepartum surveillance was carried out.The baby had no evidence of congenital heart block. She has progressive extrarticular calcification with otherwise well controlled disease and prefers to remain on IvIg. Discussion We present the first case of psoriasis with Raynaud’s developing progressive skin and soft calcification resulting in discharging sinuses. She developed myositis scleroderma overlap with suspected cardiac involvement posing challenge due to intended pregnancy. There was limited data to go by on outcomes of pregnancy in dermatomyositis and no there are similar cases in literature. In retrospect, her joint symptoms could have stemmed from extra-articular calcification around hip but does not explain skin tightening around fingers. It makes one wonder if resistant scalp psoriasis initially could be related to dermatomyositis and not true psoriasis. She was managed with regular advice from the myositis specialist unit and declined to go on any drugs which could have an impact on fertility or pregnancy. Hence options for treatment were limited and complicated by intolerance to conventional DMARDS. IvIG was selected based on those preferences due to progressive myositis but there were initial reactions to IvIG infusions at which point use of rituximab was considered. The NICE rituximab in myositis guidelines were not present at that time and the individual funding request was declined. Myositis is an idiopathic inflammatory immune mediated disorder that may be existent in an isolated form or in combination with other autoimmune or connective tissue disorders. It is a T-cell mediated cytotoxic process directed toward unknown muscle antigens. Psoriasis on the other hand is a relapsing skin disease; the diagnosis is of which is made on clinical grounds and can be associated with SpA. In a retrospective review of psoriasis patients seen at the Mayo Clinic the frequency of pathologically confirmed myopathies or inflammation in muscle in patients with psoriasis was estimated to be 0.13%. However, this could be an overestimate, given potential referral bias. Concomitant autoimmune disorders, psoriatic arthritis, and exposure to anti-TNF-α therapy were the proposed associations with increased risk of developing myopathy in psoriasis patients. Most had inclusion of body myositis. Key learning points Evidence suggests that the appropriate treatment with immunosuppressants allows a normal pregnancy without major problems and with no further risk for post-partum relapse. This is presuming the disease is well-controlled for 6 months prior to conception. There is no definite impact of pregnancy on a well-controlled myositis, although case reports have variable outcomes. Pregnancy outcomes are better if the disease is fully controlled preconception and there is no cardiac or respiratory involvement. Hence, preconception work up is done in liaison with maternal foetal medicine and includes disease activity measurements, repeat investigations for systemic involvement (commonly ECHO and pulmonary function test), repeat autoimmune screen and individualised preconception counselling. Poorly controlled disease can increase risk of intrauterine growth retardation, stillbirth or preterm birth. Uncontrolled inflammation is thought to result in poor placental circulation due to inflammatory fibrillin deposition. Autoimmune disorders are conventionally known to flare postpartum but experiences are variable. We observed a slight CK rise postpartum which settled without needing further treatment. Treatment options available for women considering pregnancy include glucocorticoids and intravenous immunoglobulin for induction of remission and remission with azathioprine, cyclosporin or tacrolimus. Data available is from case series and experiences of specialist centres only, hence there is scope for further research. Owing to limited data on the long-term use of intravenous immunoglobulins in myositis, absence of evidence-based treatment options for calcification in myositis and push for switch to rituximab due to cost implications, further management of patients in similar situations will be challenging. Conflicts of interest The authors have declared no conflicts of interest.