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Bullous pemphigoid

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https://www.readbyqxmd.com/read/28427922/-sudden-onset-bullous-pemphigoid-in-a-12-year-old-child
#1
N Ilham El Makrini, H Zaouri, O El Anzi, M Meziane, N Ismaili, L Benzekri, K Senouci, B Hassam
Bullous pemphigoid (BP) is an acquired autoimmune disease that mainly affects the elderly. It is very rare in children. We report a sudden polymorphic case of BP in a 12-year-old child.
April 17, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28419570/oral-lichenoid-reactions-associated-with-anti-pd-1-pd-l1-therapies-clinicopathological-findings
#2
V Sibaud, C Eid, V R Belum, P Combemale, B Barres, L Lamant, L Mourey, C Gomez-Roca, C L Estilo, R Motzer, E Vigarios, Mario E Lacouture
Immune checkpoint inhibitors targeting the programmed cell death receptor-1 (PD-1) or its ligand (PD-L1) show broad activity across different tumor types and currently represent one of the keystones of cancer management. Dermatologic toxicities are one of the most frequent immune-related adverse events (irAEs) induced by these new monoclonal antibodies. Maculopapular rash, pruritus, exacerbation of psoriasis or more specific autoimmune disorders (e.g. vitiligo, alopecia areata, and bullous pemphigoid) are amongst the most commonly reported AEs...
April 17, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28418613/complement-independent-blistering-mechanisms-in-bullous-pemphigoid
#3
Hiroaki Iwata, Hideyuki Ujiie
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease that clinically demonstrates tense blisters with widespread erythema, histologically demonstrates subepidermal blistering and immunologically demonstrates the presence of circulating autoantibodies against hemidesmosomal molecules. Complement activation has long been regarded as necessary for the generation of the BP. However, certain evidence has recently come to support non-complemental blistering mechanisms. The story of BP blistering mechanisms is a complicated one...
April 18, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28418589/a-distinct-cutaneous-microbiota-pofile-in-autoimmune-bullous-disease-patients
#4
Mor Miodovnik, Axel Künstner, Ewan A Langan, Detlef Zillikens, Regine Gläser, Eli Sprecher, John F Baines, Enno Schmidt, Saleh M Ibrahim
Bullous Pemphigoid (BP) is the most common autoimmune blistering disease in Europe. As both the incidence of the disease and the relative proportion of the elderly population continue to rise, it represents a significant medical burden. Whereas some progress has been achieved in defining genetic risk factors for autoimmune blistering diseases, no environmental agent has been conclusively identified. Emerging evidence suggests that host immunity may influence the skin microbiota while the latter modulates cutaneous immunity...
April 18, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28417469/oesophageal-involvement-in-26-consecutive-patients-with-mucous-membrane-pemphigoid
#5
O Zehou, J-J Raynaud, C Le Roux-Villet, M Alexandre, G Airinei, F Pascal, M Heller, N Lièvre, L Laroche, F Caux, R Benamouzig, C Prost-Squarcioni
BACKGROUND: Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described. OBJECTIVES: This study was undertaken to systematically characterise the endoscopic lesions of a series of patients with oesophageal symptoms seen in a referral centre for autoimmune bullous diseases. METHODS: Clinical, endoscopic and immunological findings of consecutively referred MMP patients with oesophageal involvement, systemic and endoscopic treatments and follow-up, are described...
April 18, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28413387/successful-treatment-of-a-bullous-pemphigoid-patient-with-rituximab-who-was-refractory-to-corticosteroid-and-omalizumab-treatments
#6
Aslı Bilgiç Temel, Cumhur Ibrahim Bassorgun, Ayşe Akman-Karakaş, Erkan Alpsoy, Soner Uzun
Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP). Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. A specific protocol for the use of rituximab to treat BP patients is not yet available. There are only small case series and case reports about the efficacy and safety of rituximab in BP...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28406394/a-randomised-controlled-trial-to-compare-the-safety-effectiveness-and-cost-effectiveness-of-doxycycline-200%C3%A2-mg-day-with-that-of-oral-prednisolone-0-5%C3%A2-mg-kg-day-for-initial-treatment-of-bullous-pemphigoid-the-bullous-pemphigoid-steroids-and-tetracyclines-blister
#7
Joanne R Chalmers, Fenella Wojnarowska, Gudula Kirtschig, James Mason, Margaret Childs, Diane Whitham, Karen Harman, Anna Chapman, Shernaz Walton, Enno Schmidt, Thomas R Godec, Andrew J Nunn, Hywel C Williams
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering skin disorder with increased morbidity and mortality in the elderly. OBJECTIVES: To evaluate the effectiveness, safety and cost-effectiveness of a strategy of initiating BP treatment with oral doxycycline or oral prednisolone. We hypothesised that starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral prednisolone...
March 2017: Health Technology Assessment: HTA
https://www.readbyqxmd.com/read/28390814/meeting-report-of-the-pathogenesis-of-pemphigus-and-pemphigoid-meeting-in-munich-september-2016
#8
Enno Schmidt, Volker Spindler, Rüdiger Eming, Masayuki Amagai, Frank Antonicelli, John F Baines, Meriem Belheouane, Philippe Bernard, Luca Borradori, Marzia Caproni, Giovanni Di Zenzo, Sergei Grando, Karen Harman, Marcel F Jonkman, Hiroshi Koga, Ralf J Ludwig, Andrew P Kowalczyk, Eliane J Müller, Wataru Nishie, Hendri Pas, Aimee S Payne, Christian D Sadik, Allan Seppänen, Jane Setterfield, Hiroshi Shimizu, Animesh A Sinha, Eli Sprecher, Michael Sticherling, Hideyuki Ujiie, Detlef Zillikens, Michael Hertl, Jens Waschke
Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases...
April 5, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28373119/photodynamic-therapy-effective-for-the-treatment-of-actinic-keratosis-and-basal-cell-carcinoma-in-bullous-pemphigoid-patients
#9
Theresa N Canavan, Salma Faghri de la Feld, Conway Huang, Naveed Sami
Treating skin cancers and extensive actinic keratosis in patients with bullous pemphigoid (BP) can be challenging. Treatment options pose unique risks in these patients as surgical wounds can have delayed wound healing and photodynamic therapy (PDT) may exacerbate their blistering disease. We report the successful use of PDT to treat actinic keratosis and skin cancers in two patients with BP, both of whom had excellent response to PDT and tolerated treatment without any bullous disease flares. Carefully selected patients with skin cancers and stable, well controlled BP can be safely considered for treatment using PDT...
March 31, 2017: Photodiagnosis and Photodynamic Therapy
https://www.readbyqxmd.com/read/28365758/amino-acid-duplication-in-the-coiled-coil-structure-of-collagen-xvii-alters-its-maturation-and-trimerization-causing-mild-junctional-epidermolysis-bullosa
#10
Jasmin K Kroeger, Silke C Hofmann, Juna Leppert, Cristina Has, Claus-Werner Franzke
The function and stability of collagens depend on the accurate triple helix formation of three distinct polypeptide chains. Disruption of this triple-helical structure can result in connective-tissue disorders. Triple helix formation is thought to depend on three-stranded coiled-coil oligomerization sites within non-collagenous domains. However, only little is known about the physiological relevance of these coiled-coil structures. Transmembrane collagen XVII, also known as 180 kDa bullous pemphigoid antigen provides mechanical stability through the anchorage of epithelial cells to the basement membrane...
February 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28355425/rituximab-treatment-of-nivolumab-induced-bullous-pemphigoid
#11
Laura Sowerby, Anna K Dewan, Scott Granter, Leena Gandhi, Nicole R LeBoeuf
No abstract text is available yet for this article.
March 29, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28352068/systemic-lupus-erythematosus-and-bullous-pemphigoid-with-dramatic-response-to-dapsone
#12
Maria Cristina Maggio, Giovanni Corsello, Eugenia Prinzi, Rolando Cimaz
BACKGROUND Bullous pemphigoid is an autoimmune blistering disease, with relapses, isolated or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Joint manifestations rapidly respond to small or moderate doses of corticosteroids, whereas skin manifestations usually respond to antimalarial drugs. CASE REPORT We describe the clinical case of an 11-year-old girl with SLE. She showed bullous skin lesions with arthralgia, mild proteinuria, resolved after steroid treatment. At the tapering of her prednisone dose, the patient had new skin lesions requiring an increased dose of prednisone...
March 29, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28351660/tweak-fn14-activation-contributes-to-the-pathogenesis-of-bullous-pemphigoid
#13
Yale Liu, Lingling Peng, Liang Li, Chengfei Liu, Xiao Hu, Shengxiang Xiao, Yumin Xia
Tumor necrosis factor-like weak inducer of apoptosis (TWEAK) participates in various cellular effects by engaging its receptor of fibroblast growth factor inducible 14 (Fn14). Increased levels of soluble TWEAK are associated with systemic autoimmunity in patients with lupus erythematosus, rheumatoid arthritis or dermatomyositis. However, the role of TWEAK in bullous pemphigoid (BP) remains unknown. In this study, we found an elevated serum level of TWEAK and a positive correlation between serum TWEAK and anti-BP180 antibodies...
March 25, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28342216/case-of-bullous-pemphigoid-coexisting-with-anti-desmoglein-autoantibodies
#14
Duerna Tie, Tokiko Yoshida, Yuko Chinuki, Xia Da, Noriyoshi Ishikawa, Eishin Morita
A 79-year-old Japanese woman had clinical and histopathological features of bullous pemphigoid, while direct immunofluorescence test revealed C3 and immunoglobulin G depositions in the lower cell surfaces of the epidermis in addition to those in the dermoepidermal junction. Chemiluminescent enzyme immunoassays were positive for desmoglein-1 and -3 antibodies in addition to anti-BP180 antibodies. In an immunoblotting study, antibodies against both 180-kDa bullous pemphigoid antigen and 130-kDa pemphigus vulgaris antigen were detected...
March 24, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28338219/human-orf-complicated-by-epidermolysis-bullosa-acquisita
#15
E Zuelgaray, C Salle de Chou, J Gottlieb, M Battistella, M D Vignon-Pennamen, M Bagot, F Guibal, J D Bouaziz
Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after Orf infection including erythema multiforme. A few cases of auto-immune bullous dermatosis complicating Orf disease have been reported to date, usually characterized by tense blisters eruptions with or without mucosal involvement, linear deposition of C3, IgG and/or IgA along the basement membrane and negativity of indirect immunofluorescence analysis and ELISA assays (performed in 4 of 11 reported cases) against target antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin-332 antibodies...
March 24, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28337472/bullous-pemphigoid-localized-in-a-primarily-hemiplegic-distribution
#16
Sean Dreyer, Maria Aleshin, Lorraine Young
No abstract text is available yet for this article.
March 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28331352/skin-disorders-in-parkinson-s-disease-potential-biomarkers-and-risk-factors
#17
REVIEW
Astrid-Helene Ravn, Jacob P Thyssen, Alexander Egeberg
Parkinson's disease (PD) is one of the most common neurodegenerative disorders, characterized by a symptom triad comprising resting tremor, rigidity, and akinesia. In addition, non-motor symptoms of PD are well recognized and often precede the overt motor manifestations. Cutaneous manifestations as markers of PD have long been discussed, and cumulative evidence shows an increased prevalence of certain dermatological disorders in PD. Seborrheic dermatitis is considered to occur as a premotor feature of PD referable to dysregulation of the autonomic nervous system...
2017: Clinical, Cosmetic and Investigational Dermatology
https://www.readbyqxmd.com/read/28329498/bullous-pemphigoid-of-infancy-report-and-review-of-infantile-and-pediatric-bullous-pemphigoid
#18
Bárbara R Ferreira, Ana S Vaz, Leonor Ramos, José P Reis, Margarida Gonçalo
A 4-month-old infant was observed with an acute itchy bullous dermatosis, predominantly involving the extremities, which revealed a dermal infiltrate rich in eosinophils, C3 deposits at the dermalepidermal junction, and circulating antibodies to BP180 antigen, confirming the diagnosis of bullous pemphigoid. He was initially treated with deflazacort 1 mg/kg/day, further increased to 2 mg/ kg/day, followed by reduction over seven weeks with complete clinical resolution within this period. We discuss epidemiology, etiology, relationship with vaccination, clinical features, and treatment of thisrelatively rare bullous dermatosis in the pediatric age...
February 16, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28323786/reply-to-direct-immunofluorescence-findings-in-discoid-lupus-erythematosus-and-bullous-pemphigoid
#19
Chika Ohata, Bungo Ohyama, Hiroshi Nagata, Minao Furumura, Takekuni Nakama
No abstract text is available yet for this article.
April 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28317060/prognostic-factors-for-mortality-in-patients-with-bullous-pemphigoid-a-meta-analysis
#20
Yi-Di Liu, Yan-Hong Wang, Yi-Cong Ye, Wen-Ling Zhao, Li Li
Bullous pemphigoid (BP) is a chronic debilitating autoimmune blistering disease that frequently occurs in the elderly population. Previous studies have suggested a high morbidity and mortality associated with BP. However, relatively few studies have investigated prognostic factors of BP mortality, and they showed considerably various results. This meta-analysis aimed to quantitatively assess the association between several potential prognostic factors and risk of mortality in bullous pemphigoid. A comprehensive search was performed using Pubmed, Embase, and Cochrane Library...
March 19, 2017: Archives of Dermatological Research
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