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Pemphigoid

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https://www.readbyqxmd.com/read/28524368/severe-pemphigoid-gestationis-associated-with-acute-disseminated-encephalomyelitis-in-the-setting-of-a-systemic-disorder
#1
Jawad Oumerzouk, Nabil Abida, Achraf Zaimi, Kaoutar Znati, El Mehdi Zbir, Ahmed Bourazza
Pemphigoid gestationis is a skin-specific autoimmune disorder that can sometimes present as the cutaneous manifestation of a multiorgan disease due to potentially common pathogenic mechanisms. We report a severe form of pemphigoid gestationis in a 32-year-old primigravida woman, who presented at 22 weeks of gestation with headaches and blurred vision, later developing encephalitis, intrauterine fetal demise and dilated cardiomyopathy.
May 19, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28520234/bullous-pemphigoid-associated-with-dipeptidyl-peptidase-4-inhibitors-a-report-of-five-cases
#2
Satoshi Yoshiji, Takaaki Murakami, Shin-Ichi Harashima, Rie Ko, Riko Kashima, Daisuke Yabe, Masahito Ogura, Kentaro Doi, Nobuya Inagaki
Bullous pemphigoid (BP) is an autoimmune blistering skin disorder. Recently, BP induced by dipeptidyl peptidase-4 (DPP-4) inhibitors has been a concern. Although DPP-4 inhibitors are commonly used in the Asian population due to their safety and efficacy, BP associated with DPP-4 inhibitors is sometimes seen in clinical settings. Here we report five Japanese cases of BP associated with the agents. In our cases, BP occurred in the elderly by 4 different DPP-4 inhibitors, which showed various clinical manifestations in terms of latency period for BP, sex, glycemic control, and diabetes duration...
May 18, 2017: Journal of Diabetes Investigation
https://www.readbyqxmd.com/read/28513499/role-of-mini-scleral-lens-in-mucous-membrane-pemphigoid
#3
Mukesh Kumar, Rohit Shetty, Chaitra Jayadev
This study aims to report the use of mini-scleral contact lens in the management of mucous membrane pemphigoid (MMP) with persistent epithelial defects. A 68-year-old male with a history of ocular pain and declining visual acuity was referred to our clinic with a diagnosis of MMP. His corrected distance visual acuity (CDVA) was 20/200 in the right eye and 20/100 in the left eye. Despite being on lubricants, topical steroids and intravenous cyclophosphamide, the patient developed persistent corneal epithelial defects in both eyes...
April 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28509683/conjunctival-squamous-cell-neoplasia-associated-with-ocular-cicatricial-pemphigoid
#4
Catherine J Choi, Frederick A Jakobiec, Fouad R Zakka, C Stephen Foster, James Chodosh, Suzanne K Freitag
The purpose of this study was to describe a possible causal relationship between ocular cicatricial pemphigoid (OCP) and ocular surface squamous neoplasia. Two middle-aged female patients with already diagnosed OCP were studied in regard to the subsequent onset of conjunctival squamous neoplasia. Their clinical histories, ocular examinations, clinical photographs, and results of biopsies and any ancillary immunofluorescent laboratory evaluation studies were carefully reviewed. One patient had a preinvasive squamous dysplasia and the other an invasive squamous cell carcinoma, both in the unequivocal setting of OCP with bilateral conjunctivitis, symblephara, and forniceal foreshortening...
May 15, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28509127/membranous-glomerulonephropathy-in-a-patient-with-bullous-pemphigoid
#5
Misa Ikeda, Hirokazu Honda, Naoto Kobayashi, Shoko Onuma, Kei Matsumoto, Tadao Akizawa, Yutaka Yamaguchi, Takanori Shibata
Bullous pemphigoid (BP) is a common autoimmune blistering disease that can be complicated by autoimmune disorders. We describe a patient with BP who developed membranous glomerulonephropathy (MN). Proteinuria decreased during the clinical course as anti-BP180 antibody titers decreased. This finding suggested an association between the pathogenesis of these two diseases in terms of immunological disorders.
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508388/positive-clinical-outcome-in-a-patient-with-recalcitrant-bullous-pemphigoid-treated-with-rituximab-and-intravenous-immunoglobulin
#6
T Nguyen, A R Ahmed
A 41-year-old white man was treated for bullous pemphigoid (BP) for 4 years, using high-dose prednisone as well as ciclosporin and mycophenolate mofetil. Sustained clinical improvement was not observed. He suffered several serious side effects. Consequently, he was treated with a combination of rituximab (RTX) and intravenous immunoglobulin (IVIg). He received 12 infusions of RTX in 6 months and monthly IVIg until the end of the therapy. Within 5 weeks of this therapy, appearance of new lesions ceased. Within 8 weeks, all previous lesions resolved and previous medications were discontinued...
May 15, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28500685/analysis-of-serum-markers-of-cellular-immune-activation-in-patients-with-bullous-pemphigoid
#7
Katja Bieber, Anna Lara Ernst, Stefan Tukaj, Maike M Holtsche, Enno Schmidt, Detlef Zillikens, Ralf J Ludwig, Michael Kasperkiewicz
Experimental models of bullous pemphigoid (BP), the most frequent subepidermal autoimmune bullous disease, revealed that the immune response leading to blister formation represents an incompletely understood complex process involving different inflammatory cells. In contrast to previous reports commonly focusing on limited molecular and cellular phenotypes of the disease, the aim of this study was to investigate a broad spectrum of markers of cellular immune activation in patients with BP. We found that serum levels of soluble CD4, myeloperoxidase, S100A12, eosinophil cationic protein, and soluble P-selectin were significantly elevated in patients with active BP compared with normal controls...
May 13, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28496445/eosinophils-in-autoimmune-diseases
#8
REVIEW
Nicola L Diny, Noel R Rose, Daniela Čiháková
Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. In this review, we summarize the function of eosinophils in inflammatory bowel diseases, neuromyelitis optica, bullous pemphigoid, autoimmune myocarditis, primary biliary cirrhosis, eosinophilic granulomatosis with polyangiitis, and other autoimmune diseases...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28494097/an-open-multicenter-randomized-clinical-study-in-patients-with-bullous-pemphigoid-comparing-methylprednisolone-and-azathioprine-with-methylprednisolone-and-dapsone
#9
M Sticherling, A Franke, E Aberer, R Gläser, M Hertl, C Pfeiffer, B Rzany, S Schneider, I Shimanovich, T Werfel, A Wilczek, D Zillikens, E Schmidt
BACKGROUND: Current treatment of bullous pemphigoid (BP) is based on the long-term use of topical and/or systemic corticosteroids associated with a high rate of adverse events and increased mortality. OBJECTIVE: To study the corticosteroid-sparing potential of azathioprine and dapsone. METHOD: A prospective, multicenter, randomized, non-blinded clinical trial that compares efficacy and safety of two parallel groups of BP patients treated with oral methylprednisolone 0...
May 11, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28487841/bullous-hemorrhagic-dermatosis-due-to-enoxaparin-use-in-a-bullous-pemphigoid-patient
#10
Ji-Su Shim, Soo Jie Chung, Byung-Keun Kim, Sae-Hoon Kim, Kyu Sang Lee, Yeonyee E Yoon, Yoon-Seok Chang
Adverse reactions of subcutaneous low molecular weight heparin or unfractionated heparin could be complications by bleeding, heparin-induced thrombocytopenia, drug-induced liver injury, osteoporosis, and cutaneous reactions. Heparin-induced skin lesions vary from allergic reactions like erythema, urticaria, eczema to intradermal microvascular thrombosis associated with heparin-induced thrombocytopenia. There is a rare cutaneous complication, called bullous hemorrhagic dermatosis. We experienced this rare case of the cutaneous complication caused by enoxaparin...
April 2017: Asia Pacific Allergy
https://www.readbyqxmd.com/read/28485892/multimorbidity-in-bullous-pemphigoid-a-case-control-analysis-of-bp-patients-with-age-and-gender-matched-controls
#11
B Sim, S Fook-Chong, Y W Phoon, H Y Koh, T Thirumoorthy, S M Pang, H Y Lee
BACKGROUND: Bullous Pemphigoid (BP) is the most common autoimmune blistering disease in the elderly and is associated with increased mortality. The extent of multimorbidity in patients with BP and its impact on survival is unclear. OBJECTIVES: To describe the extent and spectrum of multimorbidity in patients with BP and to ascertain its impact on survival. METHODOLOGY: This was a case-control study conducted in the setting of an academic medical centre...
May 9, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28485881/ist-das-bull%C3%A3-se-pemphigoid-eine-verkappte-genodermatose
#12
EDITORIAL
Heiko Traupe
No abstract text is available yet for this article.
May 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28485874/abcb1-gen-polymorphismus-in-einer-polnischen-kohorte-ist-mit-risiko-f%C3%A3-r-bull%C3%A3-ses-pemphigoid-assoziiert
#13
Mariola Rychlik-Sych, Małgorzata Barańska, Michał Dudarewicz, Jadwiga Skrętkowicz, Agnieszka Żebrowska, Jacek Owczarek, Elżbieta Waszczykowska
HINTERGRUND UND ZIELE: Polymorphismen im ABCB1-Gen, das für das P-Glykoprotein kodiert, können die intrazelluläre Konzentration von Xenobiotika beeinflussen und so zur Entwicklung von Autoimmunerkrankungen, einschließlich des bullösen Pemphigoids (BP), beitragen. In der vorliegenden Studie sollte untersucht werden, ob in einer polnischen Kohorte die C3435T- und G2677T/A-Polymorphismen im ABCB1-Gen mit dem Risiko für ein BP assoziiert sind. PATIENTEN UND METHODIK: Die Studie umfasste 71 Patienten mit BP und 156 gesunde Probanden...
May 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28479314/epiglottitis-as-the-presenting-sign-of-mucous-membranous-pemphigoid-a-case-report
#14
Narin Nard Carmel-Neiderman, Ilana Kaplan, Yael Oestreicher-Kedem
Mucous membranous pemphigoid (MMP) is an autoimmune disease of the mucous membranes characterized by formation of subepithelial blisters. MMP commonly involves the ocular and oral mucosa. Laryngeal MMP is very rare, estimated as occurring in 1 of 10 million persons in the general population. It mostly affects the supraglottis, but may also involve other laryngeal sites as well as extralaryngeal areas. This report describes the clinical picture, workup to diagnosis, and differential diagnosis of an isolated epiglottic process in an elderly female who presented with isolated, long-standing, nonresolving epiglottitis, later diagnosed as MMP with epiglottal and oral involvement...
May 4, 2017: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28468743/bullous-pemphigoid-with-igg-autoantibodies-to-the-%C3%AE-3-subunit-of-laminin-332-associated-with-psoriasis-vulgaris
#15
Masafumi Ohashi, Hajime Takagi, Yoko Mizutani, Mariko Seishima, Hiroshi Koga, Takashi Hashimoto
No abstract text is available yet for this article.
May 3, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28468735/bullous-pemphigoid-and-systemic-sclerosis-an-incidental-association
#16
Emanuele Cozzani, Margherita Cioni, Lodovica Gariazzo, Rosella Gallo, Aurora Parodi
No abstract text is available yet for this article.
May 3, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28467996/increased-risk-of-bullous-pemphigoid-after-first-ever-stroke-a-population-based-study
#17
Ai-Ling Shen, Hsiu-Li Lin, Hsiu-Chen Lin, Yuan-Fu Tseng, Chien-Yeh Hsu, Che-Yi Chou
BACKGROUND: We hypothesize that autoantibodies are induced after the blood-brain barrier is damaged by stroke and the risk of bullous pemphigoid (BP) is increased after stroke. We assess the risk of BP after first-ever stroke in a nationwide population-based cohort of first-ever stroke patients. METHODS: We extracted data from the Longitudinal Health Insurance Database 2005 and identified patients with first-ever stroke as well as control patients matched for age, gender, and year of enrollment...
May 4, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28458935/successful-management-of-acquired-hemophilia-a-associated-with-bullous-pemphigoid-a-case-report-and-review-of-the-literature
#18
Quentin Binet, Catherine Lambert, Laurine Sacré, Stéphane Eeckhoudt, Cedric Hermans
Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28456612/experimental-laminin-332-mucous-membrane-pemphigoid-critically-involves-c5ar1-and-reflects-clinical-and-immunopathological-characteristics-of-the-human-disease
#19
Eva Nina Heppe, Sabrina Tofern, Franziska S Schulze, Akira Ishiko, Atsushi Shimizu, Christian Sina, Detlef Zillikens, Jörg Köhl, Stephanie Goletz, Enno Schmidt
Mucous membrane pemphigoid (MMP) is an autoantibody-mediated disease predominantly affecting the oral cavity, pharynx, and conjunctiva. Conjunctival lesions may lead to impaired vision and finally, blindness. About 25% of MMP patients generate autoantibodies against the α3 chain of laminin 332 (LAMα3), a structural protein of epidermal/epithelial basement membranes. Here, we established a mouse model by the passive transfer of rabbit IgG against the murine homologues of two immunodominant fragments in adult C57BL/6 mice (mLAMα3)...
April 26, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28452792/epidermolysis-bullosa-acquisita-brunsting-perry-pemphigoid-variant-localized-to-the-face-and-diagnosed-with-antigen-identification-using-skin-deficient-in-type-vii-collagen
#20
Leila Asfour, Heung Chong, John Mee, Richard Groves, Manuraj Singh
Brunsting-Perry pemphigoid is defined as an autoimmune vesiculobullous eruption typically localized on the head and neck region with minimal or no mucosal involvement. The disease tends to run a chronic and recurrent course with residual scarring. Histological features are characterized by subepidermal bullae and linear IgG deposits at the dermo-epidermal junction. We report a case of a 46-year-old lady who presented with typical features of Brunsting-Perry pemphigoid. Autoantibodies to type VII collagen were identified by using recessive dystrophic epidermolysis bullosa skin which lacks type VII collagen in an indirect immunofluorescence assay...
April 12, 2017: American Journal of Dermatopathology
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