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https://www.readbyqxmd.com/read/28633746/extragenital-bullous-lichen-sclerosus-on-the-anterior-lower-extremities-report-of-a-case-and-literature-review
#1
Nichelle Arnold, Mitch Manway, Sean Stephenson, Howard Lipkin
Lichen sclerosus (LS) is a benign, chronic, inflammatory skin disease with a predilection for the anogenital region in women. Although males can also be affected, the ratio of female to male incidence has been reported to be as high as 6-10:1 and possesses a bimodal age distribution of pre-pubertal girls and postmenopausal women [1, 2]. Affected skin usually demonstrates polygonal papules that coalesce into porcelain white plaques and can be associated with edema, telangiectasias, and comedo-like plug formation [3]...
June 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28631092/rituximab-treatment-in-pemphigus-vulgaris-effect-on-circulating-tregs
#2
Bakr El-Zawahry, Dalia Bassiouny, Rehab Hegazy, Heba Gawdat, Suzan Shalaby, Mervat Khorshied, Marwah Adly Saleh
Rituximab (RTX) has been used successfully to treat refractory pemphigus. We aimed to assess the response of pemphigus vulgaris (PV) cases to RTX therapy and its effect on CD4(+)CD25(+) (T regulatory) cells level. Sixteen PV patients were included in this study, each received one cycle of two RTX infusions (1000 mg on days 1 and 15). Five PV patients served as controls. All cases were on prednisolone ± adjuvant therapy. Pemphigus disease area index (PDAI), autoimmune bullous skin intensity score (ABSIS), anti-desmoglein antibodies, CD4, CD8, CD20 and CD4(+)CD25(+) levels were assessed at baseline, 3, 6 and 12 months after therapy...
June 19, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28630891/frequency-of-neurological-disorders-in-bullous-pemphigoid-patients-a-cross-sectional-study
#3
Sheida Khosravani, Farhad Handjani, Reyhaneh Alimohammadi, Nasrin Saki
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disorder which occurs mostly in the elderly. Several studies have reported an association between BP and neurological disorders (ND). OBJECTIVE: The purpose of this study was to evaluate the association between BP and neurological disorders in Iranian patients. METHODS: In this cross-sectional study, 87 patients with BP were enrolled. They were compared to 184 controls...
2017: International Scholarly Research Notices
https://www.readbyqxmd.com/read/28628691/eruptive-melanocytic-acral-nevi-in-the-setting-of-6-mercaptopurine-therapy
#4
Arpan V Prabhu, Kristin Bibee, Joseph C English
<p>Eruptive melanocytic nevi (EMN) are a rare clinical finding characterized by sudden-onset nevi that often present in a grouped distribution. They have been associated with chemotherapy, immunosuppression, bullous diseases, and medications including multikinase and BRAF inhibitors. It is important for dermatologists to be able to identify patients with sudden development of new melanocytic nevi secondary to particular medications. Herein, we describe a case of eruptive melanocytic acral nevi secondary to 6-mercaptopurine therapy...
May 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28627382/bpag1-a-distinctive-role-in-skin-and-neurological-diseases
#5
REVIEW
Arshad Ali, Lifang Hu, Fan Zhao, Wuxia Qiu, Pai Wang, Xiaoli Ma, Yan Zhang, Lei Chen, Airong Qian
Spectraplakins are multifunctional cytoskeletal linker proteins that act as important communicators, connecting cytoskeletal components with each other and to cellular junctions. Bullous pemphigoid antigen 1 (BPAG1)/dystonin is a member of spectraplakin family and expressed in various tissues. Alternative splicing of BPAG1 gene produces various isoforms with unique structure and domains. BPAG1 plays crucial roles in numerous biological processes, such as cytoskeleton organization, cell polarization, cell adhesion, and cell migration as well as signaling transduction...
June 13, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28623936/safety-and-potential-efficacy-of-gemfibrozil-as-a-supportive-treatment-for-children-with-late-infantile-neuronal-ceroid-lipofuscinosis-and-other-lipid-storage-disorders
#6
REVIEW
Kyeongsoon Kim, Hynda K Kleinman, Hahn-Jun Lee, Kalipada Pahan
Neuronal Ceroid Lipofuscinosis (NCL), also known as Batten disease, is a group of genetically distinct lysosomal disorders that mainly affect the central nervous system, resulting in progressive motor and cognitive decline primarily in children. Multiple distinct genes involved in the metabolism of lipids have been identified to date with various mutations in this family of diseases. There is no cure for these diseases but some new therapeutic approaches have been tested that offer more hope than the standard palliative care...
June 17, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28623863/linear-immunoglobulin-a-g-bullous-dermatosis-associated-with-ulcerative-colitis
#7
Asuka Onoe, Daisuke Matsuura, Tadashi Terui, Norito Ishii, Takashi Hashimoto, Toyoko Ochiai
Linear immunoglobulin (Ig)A/G bullous dermatosis (LAGBD) is an autoimmune bullous disease characterized by formation of subepidermal blisters and linear deposition of IgA and IgG antibodies along the basement membrane zone (BMZ). The association between linear IgA bullous dermatosis and ulcerative colitis (UC) is well recognized, but reports of UC-associated LAGBD are lacking. We have reported a 24-year-old man suffering from LAGBD associated with UC, which occurred before exacerbations of skin rash. A skin biopsy indicated a subepidermal blister with an infiltration of primarily neutrophils and eosinophils in the dermis...
June 17, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28618730/possible-triggering-factors-and-comorbidities-in-newly-diagnosed-autoimmune-bullous-diseases
#8
Sevgi Akarsu, Özlem Özbağçivan, Necla Dolaş, Şebnem Aktan
BACKGROUND/AIM: The distribution and the demographic, etiological, and clinical features of autoimmune bullous diseases (ABDs) vary according to geographic regions of the world. The limited number of reported studies in Turkey are mostly retrospective and generally include only pemphigus. The aim of our study was to evaluate the demographic data, clinical features, associated triggering factors, and comorbidities in patients newly diagnosed with ABDs. MATERIALS AND METHODS: In this descriptive and prospective study, the demographic data, inhabited regions, smoking and drinking habits, presence of comorbidities, and specific and general potential triggering factors of ABDs considered by the patients were examined...
June 12, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28617149/comparison-of-airway-deposition-distributions-of-particles-in-healthy-and-diseased-workers-in-an-egyptian-industrial-site
#9
Péter Füri, Werner Hofmann, Ágnes Jókay, Imre Balásházy, Mona Moustafa, Blanka Czitrovszky, Gábor Kudela, Árpád Farkas
The objective of this study is the prediction and comparison of airway deposition patterns of an industrial aerosol in healthy workers and workers suffering from silicosis. Mass concentrations and related size distributions of particulate matter were measured in the industrial area of Samalut in Minia, Egypt. A novel stochastic lung deposition model, simulating the symptoms of silicosis by chronic bronchial (Br) obstruction and emphysema in the acinar (Ac) region, was applied to compute mass deposition fractions, deposition density, deposition rate and deposition density rate distributions in healthy and diseased workers...
June 15, 2017: Inhalation Toxicology
https://www.readbyqxmd.com/read/28611508/correlation-between-il36%C3%AE-and-il17-and-activity-of-the-disease-in-selected-autoimmune-blistering-diseases
#10
Agnieszka Żebrowska, Anna Woźniacka, Katarzyna Juczyńska, Kamila Ociepa, Elżbieta Waszczykowska, Izabela Szymczak, Rafał Pawliczak
Dermatitis herpetiformis (DH), bullous pemphigoid (BP), and pemphigus vulgaris (PV) are autoimmune bullous skin conditions with eosinophilic and neutrophilic infiltrations. While cytokines are crucial for the affinity and activation of different leukocyte cells in the inflammation and blister formation, there are no studies concerning a role of IL-36. The goal of the study was to analyze whether interleukin 36 is involved in pathogenesis of DH, BP, and PV. And the second aim of the study was the estimation of correlation between Il-36 and IL-17 and titers of specific antibodies in these diseases...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28606270/-keratoplasty-classification-and-primary-disease-spectrum-analysis-of-315-cases
#11
S B Cai, M Sun, S Li, L J Xu, W Wang, J Wang, W K Hu, X Y Li, P Wang, H Zhang, G G Li
Objective: To identify the primary disease spectrum and trends of surgical procedure of keratoplasty patients. Methods: Retrospective case series study. To review all patients who underwent keratoplasty at Department of ophthalmology in Tongji Hospital from January 1, 2012 to December 31, 2015. The data collected included age, sex, birthplace, and primary corneal disease and associated surgical procedures. Then the data were compared with similar papers domestic and foreign. Results: A total of 315 keratoplasties were performed during this 4-year period...
June 11, 2017: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/28605575/bullous-pemphigoid-and-chronic-kidney-graft-rejection
#12
F Rosique López, A Martínez Losa, P Sánchez-Pedreño Guillén, N Ishii, T Hashimoto, P Martínez García
Bullous pemphigoid (BP) is a disease caused by antibodies against components of the basement membrane, mainly two proteins: BP180 and BP230. We present two cases of BP related to chronic renal rejection. This article is protected by copyright. All rights reserved.
June 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28590036/t-regulatory-cells-and-other-lymphocyte-subsets-in-patients-with-bullous-pemphigoid
#13
T Gambichler, A Tsitlakidon, M Skrygan, S Höxtermann, L Susok, S Hessam
BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease, and is associated with autoantibodies to the hemidesmosomal BP autoantigens BPAG1 and BPAG2. AIM: We aimed to investigate the significance of T regulatory cells and other lymphocyte subsets in patients with BP. METHODS: In total, 31 inpatients with BP were treated with systemic prednisolone in a tapered dose regimen, while 28 healthy individuals matched for age and sex served as the healthy control (HC) group...
June 7, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28584371/shifting-focus-in-the-therapeutics-of-immunobullous-disease
#14
Abhishek De, Asad Ansari, Nidhi Sharma, Aarti Sarda
Therapeutics of autoimmune bullous disease has seen a major shift of focus from more global immunosuppression to targeted immunotherapy. Anti CD 20 monoclonal antibody Rituximab revolutionized the therapeutics of autoimmune bullous disease particularly pemphigus. Though it is still being practiced off-label, evidences in the form of RCT and meta analysis are now available. Other novel anti CD 20 monoclonal antibodies like ofatumumab, veltuzumab, and ocrelizumab, tositumomab or obinutuzumab/GA101 may add to the therapeutic options in coming days...
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28584369/advanced-diagnostic-techniques-in-autoimmune-bullous-diseases
#15
Anuradha Jindal, Raghavendra Rao, Balbir S Bhogal
Autoimmune blistering diseases are diverse group of conditions characterized by blisters in the skin with or without mucosal lesions. There may be great degree of clinical and histopathological overlap; hence, advanced immunological tests may be necessary for more precise diagnosis of these conditions. Direct immunofluorescence microscopy is the gold standard tests to demonstrate the tissue-bound antibodies and should be done in all cases. Magnitude of antibody level in patient' serum can be assessed by indirect immunofluorescence and enzyme linked immunosorbent assay...
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28584368/pathophysiology-of-autoimmune-bullous-diseases-nature-versus-nurture
#16
Forum Patel, Reason Wilken, Falin B Patel, Hawa Sultani, Itzel Bustos, Christopher Duong, John J Zone, Siba P Raychaudhuri, Emanual Maverakis
Pemphigus and pemphigoid are the prototypical immunobullous diseases. Although it has been well established that they are caused by deposition of autoreactive antibodies directed against adherence proteins within the skin, the specific genetic and environmental factors leading to development of these diseases continue to be an area of investigation. Herein, we discuss several of the potential environmental triggers that may induce patients to develop immunobullous diseases including medications, viral infections, UV exposure or other radiation injury and dietary factors...
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28584223/bullous-pemphigoid-and-antecedent-neurological-diseases-an-association-with-dementia
#17
Crystal Zhen Yu Phuan, Yik Weng Yew, Hong Liang Tey
BACKGROUND: Bullous pemphigoid is the most common subepidermal immunobullous disorder. Studies have reported the association between bullous pemphigoid and various neurological diseases. AIMS: The aim of this study was to evaluate whether bullous pemphigoid is associated with pre-existent neurological diseases and whether specific diseases exhibit this association. METHODS: All dermatology inpatients from January 2010 to May 2015 were analyzed...
July 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/28580800/kaposi-sarcoma-associated-with-iatrogenic-immunosuppression-a-rare-complication-of-bullous-pemphigoid-treatment
#18
Catherine Tremblay, Dominique Friedmann
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune bullous disease requiring immunosuppressive therapy. Kaposi sarcoma (KS) is an angioproliferative tumor associated with the opportunistic viral infection human herpes virus 8 (HHV-8). It is a well-known condition associated with longstanding human immunodeficiency virus infection, but it may also occur in the context of iatrogenic immunosuppression. OBJECTIVE: Although a rare complication, all dermatologists dealing with immunosuppressors must be aware and have a high index of suspicion when a patient presents with rapidly progressive violaceous papules...
June 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28580160/oxcarbazepine-induced-stevens-johnson-syndrome-a-pediatric-case-report
#19
Burçin Beken, Ceren Can, Aysegül Örencik, Nuray Can, Mehtap Yazıcıoğlu
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis are two rare but life-threatening diseases characterized by detachment of epidermis, bullous skin lesions and mucous membrane erosions. Anti-epileptic drugs are highly suspected to be the causative agents. Although carbamazepine (CBZ) is the most associated anti-epileptic drug, oxcarbazepine (OXC), which is a monohydrated derivative of CBZ, is proposed to be safer because of the different metabolism of the two drugs. Herein, we report a case of SJS induced by oxcarbazepine...
June 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28576735/the-syk-tyrosine-kinase-is-required-for-skin-inflammation-in-an-in-vivo-mouse-model-of-epidermolysis-bullosa-acquisita
#20
Tamás Németh, Oana Virtic, Cassian Sitaru, Attila Mócsai
The inflammatory form of epidermolysis bullosa acquisita is caused by autoantibodies against type VII collagen (C7), a component of the dermal-epidermal junction. We have previously shown that myeloid Src-family kinases mediate skin inflammation triggered by anti-C7 antibodies. Here we identify the Syk tyrosine kinase as a critical component of autoantibody-induced skin inflammation downstream of Src-family kinases. Immobilized C7-anti-C7 immune complexes triggered neutrophil activation and Syk phosphorylation in a Src-family kinase-dependent manner...
May 30, 2017: Journal of Investigative Dermatology
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