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scleromyxedema

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https://www.readbyqxmd.com/read/29447654/papular-mucinosis-or-localized-lichen-myxedematosis-lm-discrete-papular-type
#1
Mitalee P Christman, Kumar Sukhdeo, Randie H Kim, Shane Meehan, Evan Rieder, Kristen Lo Sicco, Andrew Franks
Lichen myxedematosus is condition characterized by localized areas of dermal deposition of mucin, presenting with firm papules localized to few areas of the body. The condition needs to be excluded from scleromyxedema, which, in addition to the firm papular eruption, has areas of induration and is usually associated with a monoclonal gammopathyand systemic symptoms. We present a 62-year-old woman with a several-year history of asymptomatic, firm papules over the face and arms with no evidence of thyroid disease or a monoclonal gammopathy,which is consistent with a diagnosis of localized lichen myxedematosus, the discrete papular variant...
December 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29368844/scleroderma-with-an-update-about-clinico-pathological-correlation
#2
Franco Rongioletti, Caterina Ferreli, Laura Atzori, Ugo Bottoni, Giuseppe Soda
Scleroderma is divided into a systemic form called systemic sclerosis and a localized form also called morphea. According to 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis, developed by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR), skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for a patient to be classified as having scleroderma. Histological examination is not included in the diagnostic criteria and is not routinely performed...
January 24, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/29283443/letter-on-european-dermatology-forum-s1-guideline-on-the-diagnosis-and-treatment-of-sclerosing-diseases-of-the-skin-part-2-scleromyxedema-scleredema-and-nephrogenic-systemic-fibrosis
#3
Patrick Mark, Ilona Dekkers, Peter Blankestijn, Tim Leiner, Giles Roditi
We read with interest the guidelines recently published on sclerosing diseases of the skin (Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis)[1, 2]. However, we are concerned that the guideline recommendations proposed for prevention of nephrogenic systemic fibrosis (NSF) are potentially dangerous. Although we recognise the challenges in constructing comprehensive guidelines, we are concerned that this may be because the guidelines have not involved a multidisciplinary team. This article is protected by copyright...
December 28, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29280826/an-unusual-cause-of-dysphagia
#4
Soumya Chatterjee, Brian J Hedman, Donald F Kirby
Scleredema adultorum of Buschke is a rare scleroderma mimic characterized by woody induration of the skin involving the back of the neck, interscapular region, upper and mid back, and sometimes the face, upper chest, and upper arms. Dysphagia is rare and not well characterized. We describe a case of scleredema that developed significant dysphagia affecting both the oropharyngeal phase and the esophageal phase of deglutition. Diagnostic evaluation revealed mild-moderate oropharyngeal and severe esophageal dysmotility...
December 27, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29228499/high-dose-intravenous-immunoglobulins-for-the-treatment-of-dermatological-autoimmune-diseases
#5
Jochen H O Hoffmann, Alexander H Enk
Based on their immunomodulatory properties, high-dose intravenous immunoglobulins (IVIGs) are successfully used in the treatment of various dermatological autoimmune diseases, in particular pemphigus vulgaris and dermatomyositis. In autoimmune bullous diseases, IVIGs can be used in an adjuvant setting (second- or third-line therapy) once combined immunosuppressive regimens have failed. In dermatomyositis, IVIGs may already be employed as an adjuvant second-line therapy after failure of corticosteroid monotherapy...
December 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28961556/interstitial-granulomatous-variant-of-scleromyxedema-a-diagnostic-pitfall
#6
Valencia Long, Weisheng Tan, Siong See Joyce Lee, Tien Guan Steven Thng
Scleromyxedema is a rare disorder where patients may develop systemic manifestations such as monoclonal gammopathy, inflammatory polyarthritis, and esophageal and neurological dysfunction. Histologically, there may be atypical variants of scleromyxedema showing features resembling interstitial granuloma annulare. We report an unusual case of scleromyxedema with interstitial granulomatous pattern and highlight potential diagnostic pitfalls when encountered with such a variant.
September 20, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28831414/paraneoplastic-atypical-scleromyxedema-with-advanced-gastric-cancer
#7
Se Jin Oh, Seung Hwan Oh, Ji-Young Jun, Ji-Hye Park, Jong Hee Lee, Dong-Youn Lee, Joo-Heung Lee, Jun-Mo Yang
No abstract text is available yet for this article.
September 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28712039/cutaneous-manifestations-of-scleroderma-and-scleroderma-like-disorders-a-comprehensive-review
#8
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...
December 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28673091/review-of-primary-cutaneous-mucinoses-in-nonlupus-connective-tissue-diseases
#9
Russell X Wong, Justin C Chia, Richard M Haber
Lichen myxedematosus is an idiopathic, cutaneous mucinosis with 2 clinicopathologic subsets. There is the generalised papular and sclerodermoid form, more properly termed scleromyxedema, and the localised papular form. We report the first case, to our knowledge, of lichen myxedematosus in association with rheumatoid arthritis as well as a case in association with dermatomyositis. An up-to-date literature review on cutaneous mucinoses and connective tissue diseases, excluding the common association of primary and secondary mucinoses with systemic lupus erythematosus, was also performed...
July 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28608042/-granulomatous-reaction-pattern-of-the-skin-interstitial-granulomatous-dermatitis-lymphoma-vasculitis
#10
REVIEW
C Rose, K Holl-Ulrich
A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Differentiating between interstitial granulomatous dermatitis and the interstitial form of granuloma annulare, early morphea and variants of borreliosis or scleromyxedema as well as interstitial granulomatous drug reaction can be very difficult...
July 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
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/28446858/a-case-of-autoimmune-polyglandular-syndrome-ype-2-associated-with-atypical-form-of-scleromyxedema
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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