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https://www.readbyqxmd.com/read/27796482/-therapeutic-administration-of-immunoglobulins
#1
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
#2
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
#3
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
#4
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...
September 25, 2016: International Journal of Dermatology
https://www.readbyqxmd.com/read/27501129/monoclonal-gammopathy-of-cutaneous-significance-review-of-a-relevant-concept
#5
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...
August 8, 2016: 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
#6
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...
July 16, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27348792/scleromyxedema-with-retinal-vasculitis-and-macular-edema
#7
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
#8
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
#9
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
#10
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
#11
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...
2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/27118492/successful-treatment-of-scleromyxedema-with-melphalan-and-dexamethasone-followed-by-thalidomide-maintenance-therapy
#12
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
#13
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
#14
Megumi Aoki, Shigeto Matsushita, Kazuhiro Kawai, Takuro Kanekura
No abstract text is available yet for this article.
January 2016: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/26897387/histopathologic-characteristics-of-scleromyxedema-a%C3%A2-study-of-a-series-of-34-cases
#15
Franco Rongioletti, Giulia Merlo, Carla Carli, Bernard Cribier, Dieter Metze, Eduardo Calonje, Werner Kempf, Catherine M Stefanato, Eduardo Marinho, Jean Kanitakis
BACKGROUND: Few histologic studies describe the histopathologic aspects of scleromyxedema. OBJECTIVE: We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients. METHODS: We studied all the cases with scleromyxedema diagnosed between 2000 and 2014 at participating centers. Sections with hematoxylin-eosin and special stains were examined. Immunohistochemistry for CD3, CD4, CD8, CD20, CD68, and factor XIIIa was performed in 10 cases...
June 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/26858056/scleromyxedema
#16
Ying Yang, Hao Chen, Wei Zhang, Jian-Fang Sun
No abstract text is available yet for this article.
February 9, 2016: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/26806503/-monoclonal-immunoglobulin-m-ig-and-skin-diseases-from-the-group-of-mucinoses-scleredema-adultorum-buschke-and-scleromyxedema-description-of-four-cases-and-an-overview-of-therapies
#17
Zdeněk Adam, Petr Szturz, Marta Krejčí, Vladimír Vašků, Luděk Pour, Eva Michalková, Sabina Ševčíková, Zdeňka Čermáková, Karel Veselý, Jíří Vaníček, Eva Pourová, Zdeněk Král, Jiří Mayer
INTRODUCTION: The mucinoses of the type of scleredema and scleromyxedema are diseases marked by excessive production of mucin deposits in the skin and subcutaneous tissue, which causes skin hardening. The skin and subcutaneous deposits hamper the movement of limbs, the thorax as well as mouth. The same mechanism also damages other organs (the heart, lungs, oesophagus). It is probably caused by the stimulation of mucin production in fibroblasts by immunoglobulins, frequently monoclonal immunoglobulin...
December 2015: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/26754417/fatal-recurrent-dermatoneuro-syndrome-associated-with-systemic-al-amyloidosis
#18
Lea Leonardis, Dominika Novak Pihler, Matija Zupan, Alenka Vizjak, Neza Lebic Belcijan, Vesna Jurcic, Mara Popovic
A male patient is presented with long-lasting paraproteinemia of monoclonal IgG λ, who suffered from recurrent, and until the last one, mostly reversible episodes of dermatoneuro syndrome, described exclusively in scleromyxedema. The skin biopsy revealed λ-light chain amyloid deposition instead of changes typical for scleromyxedema. Systemic AL amyloidosis was diagnosed post mortem since the patient had no clinical signs of any other organ impairment except skin and brain. Neuropathology is described and possible etiopathogenesis of brain involvement is considered...
March 2016: Clinical Neuropathology
https://www.readbyqxmd.com/read/26676919/frequency-of-cutis-laxa-like-clinical-features-and-elastolysis-in-scleromyxedema-a-retrospective-clinicopathologic-study-of-19-patients-with-scleromyxedema
#19
Tania M Gonzalez Santiago, Julia S Lehman, J Noelle Buonaccorsi, Amer N Kalaaji, Carilyn N Wieland
BACKGROUND: Cutis laxa-like features were observed in a subset of patients with scleromyxedema. Given this observation, clinical and histopathologic features of scleromyxedema were reviewed in correlation with elastic tissue staining. METHODS: We retrospectively reviewed clinical records and histopathologic features from patients with scleromyxedema seen at our institution from 1992 through 2013. We also evaluated available skin biopsies with an elastin stain and assessed whether dermal elastin fibers were diminished in density or were fragmented (or both)...
March 2016: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/26437283/scleromyxedema-secondary-to-hepatitis-c-virus-and-successfully-treated-with-antiviral-therapy
#20
J A Smith, F A Kalimullah, C P Erickson, L S Peng
Scleromyxedema (SM) is a chronic and progressive fibromucinous disease with no known etiology. We report a patient with scleromyxedema associated with hepatitis C virus, successfully treated with interferon and ribavirin therapy.
September 17, 2015: Dermatology Online Journal
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