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scleromyxedema

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https://www.readbyqxmd.com/read/28446858/a-case-of-autoimmune-polyglandular-syndrome-ype-2-associated-with-atypical-form-of-scleromyxedema
#1
Oleksander Prylutskyi, Olga Prylutska, Anatoliy Degonskyi, Kseniia Tkachenko
BACKGROUND: Autoimmune polyglandular syndrome type 2 represents an uncommon endocrine disorder composed by Addison's disease with autoimmune thyroid disease (Schmidt's syndrome) and/or type 1 diabetes mellitus. Scleromyxedema is a rare progressive cutaneous mucinosis usually associated with systemic involvement and paraproteinemia. To the best of our knowledge, there is no case report of Schmidt's syndrome associated with scleromyxedema. CASE DETAILS: A 34-year-old woman was admitted to Donetsk Clinical Territorial Medical Association due to acute general weakness, reduced vision, dryness of integuments, memory decline, fatigue, weight loss, rash on the face trunk and extremities...
September 2016: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/28370513/high-dose-intravenous-immunoglobulin-therapy-for-scleromyxoedema-a-prospective-open-label-clinical-trial-using-an-objective-score-of-clinical-evaluation-system
#2
A Guarneri, M Cioni, F Rongioletti
BACKGROUND: Scleromyxoedema is a primary fibro-mucinosis whose therapy is still challenging. OBJECTIVE: To evaluate the safety and efficacy of high-dose intravenous immunoglobulin (IVIg) for the management of scleromyxoedema prospectively using an objective score. METHODS: In a prospective open-label study, IVIg was administered to eight patients with scleromyxoedema in a dose of 2 g/kg per month. The patients were followed-up to a minimum of 6 months, and their disease activity and response to treatment were assessed using the Physician's Global Assessment of disease severity (PGA) and a modified objective skin scoring system for patients with scleroderma (modified Rodnan score system for scleromyxoedema or mRSSS)...
March 29, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28300892/scleromyxedema-clinical-diagnosis-and-autopsy-findings
#3
Ana Carolina Bulhões Sala, Paulo Rowilson Cunha, Clóvis Antônio Lopes Pinto, Célia Antônia Xavier de Moraes Alves, Ingrid Barreto Paiva, Ana Paula Vieira Araujo
Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28217476/a-case-of-scleromyxedema-responding-to-lenalidomide-and-dexamethasone
#4
Sweta Rambhia, Kinjal Rambhia, Amit Gulati, Nirmal Raut
No abstract text is available yet for this article.
January 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/28099617/case-for-diagnosis-lichen-myxedematosus
#5
Priscila Regina Orso Rebellato, Mauren Beatriz Frazon Carbonar, Nicole Iasmin Magario Tabuti, Graziela Junges Crescente Rastelli
Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy...
November 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27990110/scleromyxedema-without-paraproteinemia-treatment-with-thalidomide-and-prednisolone
#6
Sara Saniee, Ghazaleh Davarnia
Scleromyxedema is a rare and distinctive variant of cutaneous mucinoses of unknown etiology. It is presenting with generalized papular eruption and sclerodermoid induration. Numerous treatment modalities have been reported to produce partial or permanent responses. This study reports on a case of scleromyxedema without paraproteinemia in a subject who experienced a partial response to thalidomide and prednisolone.
September 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/27796482/-therapeutic-administration-of-immunoglobulins
#7
REVIEW
T Witte
BACKGROUND: Intravenously administered immunoglobulins have multiple modes of action that are anti-inflammatory. They can therefore be beneficial in a number of autoimmune disorders. OBJECTIVE: The aim of this article is to analyze and summarize studies on the administration of intravenous immunoglobulins in rheumatological diseases. METHODS: A selective search and analysis of the literature was carried out related to the mode of action and efficacy of intravenous immunoglobulins in rheumatological diseases...
December 2016: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/27708955/dermato-neuro-syndrome-in-a-case-of-scleromyxedema
#8
Yusuf Savran, Sevgi Akarsu
Scleromyxedema is an uncommon connective tissue disease characterized by mucin deposits, fibrosis, and proliferation of fibroblasts in the dermis. Although it shares similar sclerodermoid features, it is a different clinical entity than scleroderma. A monoclonal gammopathy is almost always present; however, progression to multiple myeloma is rare. It may have many systemic manifestations, of which the most notable being the dermato-neuro syndrome because of its rarity and potential fatal outcome. We present a case of a 50-year-old woman with scleromyxedema in whom the dermato-neuro syndrome developed...
December 2015: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27698568/atypical-scleromyxedema-presenting-with-cutaneous-and-cardiovascular-manifestations
#9
Sue-Ann Teh, David A Kandiah
Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen. This condition can be localized as discrete papular lichen myxedematous skin or as a systemic condition usually associated with paraproteinaemia. To date, there is no unifying treatment and is limited by rarity, small number of case reports, and the lack of randomized controlled trials. We describe the case of a 56-year-old gentleman with features of scleromyxedema who had cutaneous and cardiac involvement, and significant mediastinal lymphadenopathy without monoclonal gammopathy...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27667657/lichen-myxedematosus-diagnostic-criteria-classification-and-severity-grading
#10
Ahmad Nofal, Hala Amer, Rania Alakad, Eman Nofal, Fatma El Desouky, Ayman Yosef, Waleed Albalat, Khaled Gharib, Inass Mostafa, Sahar Fathy, Monira Waked, Eman Ragheb, Hanan Gammaz
BACKGROUND: Lichen myxedematosus (LM) is a rare, chronic idiopathic disorder characterized clinically by waxy, closely set papules and histopathologically by diffuse dermal mucin deposition and fibroblast proliferation. The most recent classification of LM was proposed in 2001; however, it seems to be complex, confusing, and imprecise. Herein, we present seven cases of LM to evaluate the validity of the current classification, to propose new diagnostic criteria and classification, and to suggest a clinically relevant severity grading system for this rare disorder...
March 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/27501129/monoclonal-gammopathy-of-cutaneous-significance-review-of-a-relevant-concept
#11
REVIEW
D Lipsker
Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided. Amyloidosis, macroglobulinoderma and follicular hyperkeratotic spicules result from extravascular immunoglobulin or immunoglobulin-related protein deposition. Skin findings include papules and plaques, follicular spicules, purpura, haemorrhagic bullae, macroglossia and nail changes...
January 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27422288/atypical-discrete-papular-lichen-myxedematosus-associated-with-monoclonal-gammopathy-report-of-four-cases-and-review-of-the-literature
#12
REVIEW
C Hermans, I Goldscheider, T Ruzicka, F Rongioletti
BACKGROUND: Discrete papular lichen myxedematosus (DPLM) is a rare form of localized lichen myxedematosus that presents with skin involvement only and without systemic involvement. OBJECT: To describe our experience with atypical cases of DPLM associated with monoclonal gammopathy. METHODS: Data were collected from patients with clinicopathological evidence of DPLM associated with monoclonal gammopathy who presented to the Department of Dermatology of two tertiary university-affiliated medical centres from 2000 to 2015 and were followed prospectively...
December 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27348792/scleromyxedema-with-retinal-vasculitis-and-macular-edema
#13
Karen I Gutiérrez-Aguirre, Michael W Stewart, Jason C Sluzevich
BACKGROUND/PURPOSE: To describe the findings and clinical course of a patient with scleromyxedema complicated by retinal vasculitis and macular edema. METHODS: Interventional case report. RESULTS: A 64-year old Caucasian woman with recently diagnosed, biopsy proven scleromyxedema presented with decreased visual acuity (20/50 OD; 20/100 OS) due to retinal vasculitis and macular edema. She received intravitreal bevacizumab and subtenon's triamcinolone acetonide for the macular edema, and bilateral pars plana vitrectomies were required for vitreous hemorrhages...
June 24, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/27294055/a-rare-association-of-scleromyxedema-with-cutis-verticis-gyrata
#14
Savita Koregol, Ramesh V Yatagiri, Sidramappa R Warad, Nasiruddin R Itagi
Cutis verticis gyrata (CVG) is characterized by redundant skin on the scalp that exhibits deep furrows and convolutions. We report a 58-year-old male who presented with multiple raised asymptomatic skin-colored lesions over the chest and abdomen and features of CVG. Biopsy from the lesion confirmed the diagnosis of scleromyxedema. Scleromyxedema presents as diffuse thickening of the skin which underlies the papules. The hypertrophy and folding of the skin produces a gyrate appearance.
May 2016: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/27282303/neurological-involvement-in-the-course-of-scleromyxedema-a-case-report
#15
Francesca Spagnolo, Cecilia Nozzoli, Augusto Rini, Salvatore La Spada, Vincenzo De Marco, Bruno Passarella
Scleromyxedema is a rare disease of unknown etiology primarily affecting the skin, characterized by generalized papular eruption, dermal fibroblast proliferation with mucin deposition, and a monoclonal gammopathy. Neurological impairment is a rare but sometimes fatal complication of scleromyxedema that should be rapidly identified to prevent significant morbidity and mortality. A 63-year-old Caucasian man had a 2-year history of scleromyxedema, and was under immunosuppressive treatment with ciclosporine and methotrexate...
September 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27207484/scleromyxedema-long-term-follow-up-after-high-dose-melphalan-with-autologous-stem-cell-transplantation
#16
Ramya Chockalingam, Madeleine Duvic
BACKGROUND: Scleromyxedema is a cutaneous mucinosis that is often accompanied by severe systemic manifestations. New therapeutic options have been introduced for this condition, but data on the long-term efficacy of treatments are limited. OBJECTIVES: This study was designed to evaluate the long-term efficacy and safety of treatment with high-dose melphalan and autologous peripheral blood stem cell transplantation (PBSCT). METHODS: In an original study published in 2006, seven patients with scleromyxedema were treated with high-dose melphalan and autologous PBSCT between April 2000 and November 2003...
October 2016: International Journal of Dermatology
https://www.readbyqxmd.com/read/27136633/correspondence-the-association-between-morphea-profunda-and-monoclonal-gammopathy-a-case-series
#17
LETTER
Justin Endo, Nicole Strickland, Simer Grewal, Travis Vandergriff, Thomas Keenan, B Jack Longley, Heidi Jacobe
It is known that eosinophilic fasciitis can be associated with monoclonal gammopathy. There is clinical similarity between eosinophilic fasciitis and morphea profunda, but it is unclear whether morphea profunda might be associated with monoclonal gammopathy. The temporal quantification of gammopathy in morphea profunda has not been well characterized. We describe four patients with morphea profunda that were associated with monoclonal gammopathy. Three were associated with monoclonal IgG protein and one with IgM...
March 16, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/27118492/successful-treatment-of-scleromyxedema-with-melphalan-and-dexamethasone-followed-by-thalidomide-maintenance-therapy
#18
Xin-Xin Cao, Tao Wang, Yue-Hua Liu, Dao-Bin Zhou, Jian Li
No abstract text is available yet for this article.
April 27, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/26969478/-atypical-scleromyxedema-with-a-granulomatous-histological-pattern-and-delayed-sclerosis
#19
G de Cambourg, R Goussot, C Wettlé, B Cribier
BACKGROUND: Papular mucinosis is characterised by primary mucin deposition in the dermis. The classification distinguishes between the localised form and the systemic form, which alone can result in complications, but this classification occasionally proves to be inadequate. Herein we report the progression of papular mucinosis, initially atypical due to the absence of cutaneous sclerosis and of misleading granulomatous histological features, which subsequently developed into characteristic scleromyxedema...
May 2016: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/26951784/scleromyxedema-clinically-resembling-to-scleroderma
#20
Megumi Aoki, Shigeto Matsushita, Kazuhiro Kawai, Takuro Kanekura
No abstract text is available yet for this article.
January 2016: Indian Journal of Dermatology
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