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Eosinophilic fasciitis

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https://www.readbyqxmd.com/read/29108116/arthritis-in-melorheostosis-an-uncommon-feature-in-a-rare-disease
#1
Enrico Selvi, Marco Bardelli, Giacomo Maria Guidelli
A 53-year-old male developed left knee and left ankle arthritis associated with marked induration of the skin and soft tissues distal to the knees. The left knee was swollen, tender, and with a non-reducible contracture and similarly the left ankle and the subtalar joint were stiff and moderately swollen. In addition, diffuse, bluish, small vascular ectasia, hypertrichosis and skin pigmentation of the affected sites were evident (fig1). These symptoms began 20 years before and the patient had been diagnosed with eosinophilic fasciitis and treated with daily prednisone leading to partial improvement of the symptoms, but not of the knee deformity and soft tissue induration...
November 6, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29101481/eosinophilic-fasciitis-an-updated-review-on-diagnosis-and-treatment
#2
REVIEW
Daniel R Mazori, Alisa N Femia, Ruth Ann Vleugels
PURPOSE OF REVIEW: First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF. RECENT FINDINGS: Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response...
November 4, 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29074338/-18-f-fdg-pet-ct-may-be-a-useful-adjunct-in-diagnosis-of-eosinophilic-fasciitis
#3
Javier Narváez, Pablo Juarez, Isabel Morales Ivorra, Laura Rodriguez Bel, Jesus Rodriguez Moreno, Montserrat Romera
No abstract text is available yet for this article.
October 23, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28967434/-sarcoid-like-granulomatosis-associated-with-eosinophilic-fasciitis
#4
E Zuelgaray, C Sallé de Chou, M-D Vignon-Pennamen, M Battistella, S Leonard-Louis, L Hefez, F Guibal, M Bagot, J-D Bouaziz
BACKGROUND: Eosinophilic fasciitis (EF) is a rare condition characterized by swelling of the extremities, sclerodermatous evolution and frequent hypereosinophilia. Hematological disorders, including aplastic anemia, solid tumors and autoimmune diseases, may be associated with EF. EF is usually not associated with granulomatous diseases. CASE REPORT: Herein we describe the case of an 80-year-old man with symmetrical swelling and sclerosis of the legs, typical of EF, associated with skin and lymph node granulomas...
September 26, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28934835/positive-prayer-sign-in-eosinophilic-fasciitis
#5
José Pardos-Gea
No abstract text is available yet for this article.
April 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28867532/-eosinophilia-heralding-the-diagnosis-of-eosinophilic-fasciitis-shulman-s-disease
#6
V Ernest, N Sautereau, E Masson, D Chemouni, M Garcia, J Bertolino, P Rossi, B Granel
INTRODUCTION: Eosinophilic fasciitis or Shulman's disease is characterized, in its typical form, by palpable thickening of the skin and soft tissues, blood hypereosinophilia and fascia lesions. We hereby report a case of eosinophilic fasciitis in which hypereosinophilia preceded for several months the clinical signs of fasciitis. CASE REPORT: A 64-year-old woman, with a history of Little's syndrome with motor disability, was admitted in internal medicine for eosinophilia...
August 31, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28865864/clinicopathologic-and-immunophenotypic-features-of-eosinophilic-fasciitis-and-morphea-profunda-a-comparative-study-of-27-cases
#7
Oluwakemi Onajin, Carilyn N Wieland, Margot S Peters, Christine M Lohse, Julia S Lehman
BACKGROUND: Eosinophilic fasciitis (EF) and morphea profunda (MP) are inflammatory and sclerosing disorders of the subcutis that can exhibit clinical and pathologic presentations that overlap. OBJECTIVE: To identify clinicopathologic features that can be used to distinguish EF from MP. METHODS: We performed a retrospective review of 16 patients with EF and 11 patients with MP. Hematoxylin-eosin, CD123, CD34, and Verhoeff-Van Gieson stains were evaluated on skin biopsies that included the fascia...
August 30, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28760502/-extensive-connective-tissue-nevus-in-children
#8
O Lafargue, S Fraitag, O Boccara, F Comoz, J Rod, C Turgis Mezerette, A Dompmartin
BACKGROUND: Connective tissue nevus (CTN) is a rare condition of the extracellular matrix components that generally presents as papulae of normal skin colour. This condition may be syndromic or sporadic. PATIENTS AND METHODS: We report herein two isolated cases of extensive and infiltrative CTN in children at risk for subsequent joint stiffening. The pathology samples displayed respectively mixed hamartoma and a collagenoma. DISCUSSION: The onset of these lesions is often difficult to establish, since they are usually unnoticeable at first...
November 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28734566/long-term-outcome-of-eosinophilic-fasciitis-a-cross-sectional-evaluation-of-35-patients
#9
Jorre S Mertens, Rogier M Thurlings, Wietske Kievit, Marieke M B Seyger, Timothy R D Radstake, Elke M G J de Jong
BACKGROUND: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course. OBJECTIVE: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up. METHODS: Patients with biopsy-proven EF were included for this cross-sectional study. Outcome measures included the Physician's Global Assessment of Disease Activity, Physician's Global Assessment of Damage (PhysGA-D), skin pliability scores, passive range of motion, and health-related quality of Life (HRQoL) questionnaires...
September 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28712039/cutaneous-manifestations-of-scleroderma-and-scleroderma-like-disorders-a-comprehensive-review
#10
REVIEW
Caterina Ferreli, Giulia Gasparini, Aurora Parodi, Emanuele Cozzani, Franco Rongioletti, Laura Atzori
Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations...
July 16, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28606036/scleroderma-like-disorders
#11
Amit Sharma
Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. These are termed as "scleroderma variants" or "scleroderma like disorders"...
June 11, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28528611/eosinophilic-fasciitis-a-firm-warning
#12
Warren R Heymann
No abstract text is available yet for this article.
2017: Skinmed
https://www.readbyqxmd.com/read/28477272/hif-1%C3%AE-mdm2-cdk4-and-p16-expression-in-ischemic-fasciitis-focusing-on-its-ischemic-condition
#13
Yuichi Yamada, Izumi Kinoshita, Kenichi Kohashi, Hidetaka Yamamoto, Yuki Kuma, Takamichi Ito, Kenji Koda, Atsushi Kisanuki, Manabu Kurosawa, Michiko Yoshimura, Masutaka Furue, Yoshinao Oda
Ischemic fasciitis is a benign myofibroblastic lesion, occurring in the sacral region or proximal thigh of elderly or bedridden individuals. The pathogenesis of ischemic fasciitis is thought to be based on ischemic condition; however, it has never been demonstrated. In this study, we examined the expression of ischemia-associated proteins in ischemic fasciitis by immunohistochemical and genetic methods. Specifically, this study aimed to reveal the expression of HIF-1α, MDM2, CDK4, p16, and gene amplification of MDM2 gene...
July 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28440714/hypereosinophilic-syndrome-endomyocardial-biopsy-versus-echocardiography-to-diagnose-cardiac-involvement
#14
COMPARATIVE STUDY
Joseph H Butterfield, Garvan C Kane, Catherine R Weiler
OBJECTIVE: To compare echocardiograms and endomyocardial biopsies to diagnose cardiac involvement in hypereosinophilic syndrome. METHODS: We examined the agreement between echocardiography and endomyocardial biopsies to detect cardiac involvement in hypereosinophilic syndrome by reviewing cases identified as hypereosinophilia or hypereosinophilic syndrome in Mayo Clinic databases from January 1978 through June 2009. Single-organ cases of eosinophilia such as eosinophilic fasciitis and eosinophilic gastroenteritis were excluded...
June 2017: Postgraduate Medicine
https://www.readbyqxmd.com/read/28393380/th1-and-th17-polarized-immune-infiltrates-in-eosinophilic-fasciitis-a-potential-marker-for-histopathologic-distinction-from-morphea
#15
Andrea Primiani Moy, Elena Maryamchik, Olga V Nikolskaia, Rosalynn M Nazarian
BACKGROUND: Morphea (localized scleroderma) and eosinophilic fasciitis (EF) are rare fibrosing disorders which may present a diagnostic challenge. While histopathologic features are often distinct, in some cases there may be overlap. T-cells contribute to etiopathogenesis of both autoimmune conditions. We sought to determine whether T-cell immune polarization enables histopathologic distinction. MATERIALS & METHODS: We retrospectively examined clinicopathologically confirmed cases of morphea (n = 12) and EF (n = 8) using immunohistochemistry for CD3, CD8, and dual staining for CD4 with T-bet, GATA-3, STAT-3 or BNC-2 (transcription factors reported to be specific and mutually exclusive for Th1, Th2, Th17 and Th22 cells, respectively) to characterize the T-cell infiltrate...
June 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28379209/diagnostic-utility-of-magnetic-ressonance-imaging-in-eosinophilic-fasciitis
#16
Alexandra Daniel, Gisela Eugénio, Sara Serra, Armando Malcata, Maria João Salvador
No abstract text is available yet for this article.
July 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28303481/morphea-and-eosinophilic-fasciitis-an-update
#17
REVIEW
Jorre S Mertens, Marieke M B Seyger, Rogier M Thurlings, Timothy R D J Radstake, Elke M G J de Jong
Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated...
August 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28300895/eosinophilic-fasciitis
#18
Elisa Baranski Lamback, Fernanda Simões Seabra Resende, Thiara Cristina Rocha Lenzi
Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with a variable infiltration of eosinophils. Peripheral eosinophilia, poly or monoclonal hypergammaglobulinemia and increased erythrocyte sedimentation rate can be seen. Clinical features begin acutely, with local edema and a painful and symmetrical stiffening of the limbs, progressing rapidly to fibrosis, which can limit joint movements. Some cases have a history of strenuous physical exercise or trauma...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28277348/groove-sign-of-eosinophilic-fasciitis
#19
Renee Fruchter, Daniel R Mazori, Alisa N Femia
No abstract text is available yet for this article.
April 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28150344/a-case-of-eosinophilic-fasciitis-associated-with-pyoderma-gangrenosum
#20
LETTER
E L Maranda, R Sheinin, A Brys, B Rubin, H W Lim
No abstract text is available yet for this article.
February 1, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
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