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Bullous pemphigoid

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https://www.readbyqxmd.com/read/29767490/kaposi-s-sarcoma-associated-with-localized-bullous-pemphigoid-two-conflicting-diseases
#1
Lucia Brambilla, Athanasia Tourlaki, Simona Tavecchio
No abstract text is available yet for this article.
August 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/29760835/cutaneous-mucormycosis-following-a-bullous-pemphigoid-flare-in-a-chronic-lymphocytic-leukemia-patient-on-ibrutinib
#2
Matthew K Stein, Saradasri Karri, Jackson Reynolds, Jeff Owsley, Austin Wise, Mike G Martin, Fereshteh Zare
While the recent development of novel therapeutics in oncology, such as small molecule kinase inhibitors (SMKIs), has enabled our ability to target disease-specific molecular pathways, the prolonged impact of these agents on the immune system and infectious risk remains to be seen. We present a 68-year-old male with refractory chronic lymphocytic leukemia (CLL) on ibrutinib monotherapy for 3 years who developed extensive cutaneous mucormycosis following a severe bullous pemphigoid (BP) flare. He received amphotericin B for 4 weeks and was continued on posaconazole with resolution of his mucormycosis infection...
April 2018: World Journal of Oncology
https://www.readbyqxmd.com/read/29756688/localized-genital-bullous-pemphigoid
#3
S J Mounsey, K Heelan, S Hughes, H Fawcett, C B Bunker
Genital bullous pemphigoid (GBP) is a rare localized subset of bullous pemphigoid (BP). BP is characterized by autoantibodies against hemidesmosomes, which are involved in the structural integrity of the epidermis, and this results in subepidermal blistering. Typically, GBP affects women and children. We report an adult male who presented with a scrotal rash and blisters that developed into erosions. Only two previous cases in men have been reported. Immunofluoresence and histopathology confirmed the diagnosis of BP...
May 14, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29737533/a-randomized-first-in-human-healthy-volunteer-trial-of-bivv009-a-humanized-antibody-for-the-specific-inhibition-of-the-classical-complement-pathway
#4
Johann Bartko, Christian Schoergenhofer, Michael Schwameis, Christa Firbas, Martin Beliveau, Colin Chang, Jean-Francois Marier, Darrell Nix, James C Gilbert, Sandip Panicker, Bernd Jilma
Aberrant activation of the classical complement pathway is the common underlying pathophysiology of orphan diseases such as bullous pemphigoid, antibody-mediated rejection of organ transplants, cold agglutinin disease and warm autoimmune haemolytic anaemia. Therapeutic options for these complement-mediated disorders are limited and BIVV009, a humanized monoclonal antibody directed against complement factor C1s, may be potentially useful for inhibition of the classical complement pathway. A phase-1, first-in-human, double-blind, randomized, placebo-controlled, dose-escalation trial of single and multiple doses of BIVV009 or placebo was conducted in 64 volunteers to evaluate safety, tolerability, pharmacokinetic, and pharmacodynamic profiles...
May 8, 2018: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29729120/autoimmune-bullous-diseases-in-non-hiv-kaposi-s-sarcoma-a-retrospective-study-in-a-large-cohort-of-patients
#5
A Tourlaki, G Genovese, E Guanziroli, B M Scoppio, E Berti, L Brambilla
BACKGROUND: Kaposi's sarcoma (KS) is a rare endothelial neoplasm caused by the human herpes virus 8 (HHV-8). Its risk is increased in immunocompromised patients, including those undergoing immunosuppressive therapy for autoimmune bullous diseases. Conversely, HHV-8 infection has been hypothesized to be a triggering factor of bullous diseases, especially pemphigus. Given the fact that both KS and autoimmune bullous diseases have a low incidence in the general population, it could be expected that the association between these disorders would be exceptional...
May 5, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29719538/humoral-epitope-spreading-in-autoimmune-bullous-diseases
#6
REVIEW
Dario Didona, Giovanni Di Zenzo
Autoimmune blistering diseases are characterized by autoantibodies against structural adhesion proteins of the skin and mucous membranes. Extensive characterization of their autoantibody targets has improved understanding of pathogenesis and laid the basis for the study of antigens/epitopes diversification, a process termed epitope spreading (ES). In this review, we have reported and discussed ES phenomena in autoimmune bullous diseases and underlined their functional role in disease pathogenesis. A functional ES has been proposed: (1) in bullous pemphigoid patients and correlates with the initial phase of the disease, (2) in pemphigus vulgaris patients with mucosal involvement during the clinical transition to a mucocutaneous form, (3) in endemic pemphigus foliaceus, underlining its role in disease pathogenesis, and (4) in numerous cases of disease transition associated with an intermolecular diversification of immune response...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29706950/bullous-pemphigoid-triggered-by-thermal-burn-under-medication-with-a-dipeptidyl-peptidase-iv-inhibitor-a-case-report-and-review-of-the-literature
#7
Yosuke Mai, Wataru Nishie, Kazumasa Sato, Moeko Hotta, Kentaro Izumi, Kei Ito, Kazuyoshi Hosokawa, Hiroshi Shimizu
Bullous pemphigoid (BP) is a common autoimmune blistering disease in which autoantibodies mainly target the hemidesmosomal component BP180 (also known as type XVII collagen) in basal keratinocytes. Various triggering factors are known to induce BP onset, including radiotherapy, burns, ultraviolet exposure, surgery, and the use of dipeptidyl peptidase-IV inhibitors (DPP4i), which are widely used antihyperglycemic drugs. Here, we present a case of BP triggered by a thermal burn under medication with DPP4i. A 60-year-old man with type II diabetes had been treated with the DPP4i linagliptin for 1 year...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29704595/treg-deficiency-leads-to-pathogenic-bp230-autoantibody-and-autoimmune-bullous-disease
#8
Stefanie Haeberle, Xiaoying Wei, Katja Bieber, Stephanie Goletz, Ralf J Ludwig, Enno Schmidt, Alexander H Enk, Eva N Hadaschik
BACKGROUND: Autoimmune bullous dermatoses (AIBD) are severe autoantibody-mediated skin diseases. The pathogenic relevance of autoreactive CD4+ T cells for the induction of autoantibody production remains to be fully evaluated. Scurfy mice lack functional regulatory T cells (Treg), develop spontaneous activation of autoreactive CD4+ T cells and display severe erosive skin lesions suggestive of AIBD. OBJECTIVE: To determine whether AIBD develop in Treg-deficient scurfy mice...
April 25, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29704593/treg-dysfunction-induces-autoantibodies-to-bullous-pemphigoid-antigens-in-mice-and-humans
#9
Ken Muramatsu, Hideyuki Ujiie, Ichiro Kobayashi, Wataru Nishie, Kentaro Izumi, Takamasa Ito, Norihiro Yoshimoto, Ken Natsuga, Hiroaki Iwata, Hiroshi Shimizu
BACKGROUND: Regulatory T cells (Tregs) play a crucial role in peripheral immune tolerance in multiple organs, including the skin. Thus far, the impact of peripheral immune tolerance failure on autoantibody-related autoimmune reaction to the skin is unclear. OBJECTIVE: We sought to elucidate the target autoantigens in the skin under the condition of Treg dysfunction due to Foxp3 gene mutations in scurfy mice and patients with immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome...
April 25, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29693698/eosinophils-are-a-major-source-of-interleukin-31-in-bullous-pemphigoid
#10
Urda Rüdrich, Manuela Gehring, Eleni Papakonstantinou, Anja Rabenhorst, Judith Engmann, Alexander Kapp, Karin Hartmann, N Helge Meyer, Bernhard F Gibbs, Ulrike Raap
Bullous pemphigoid (BP) is characterized by substantial skin and blood eosinophilia as well as intensive pruritus. Since the pruritogenic cytokine interleukin (IL)-31 is increased in inflammatory skin diseases the aim of this study was to determine whether IL-31 plays a role in BP. Using immunofluorescence, IL-31 expression was analysed in eosinophils derived from blister fluids and skin from patients with BP and IL-31 levels in blister fluids, serum and culture supernatants were determined by enzyme-linked immunoassay (ELISA)...
April 24, 2018: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29693068/explosive-bullous-pemphigoid-with-high-serum-total-ige-serum-ige-as-a-biomarker-that-reflects-disease-activity
#11
Hyun Jung Kwon, Ga Ram Ahn, Sun Young Choi, Kapsok Li, Seong Jun Seo
No abstract text is available yet for this article.
May 2018: JAAD Case Reports
https://www.readbyqxmd.com/read/29686678/immunoglobulin-e-mediated-autoimmunity
#12
REVIEW
Marcus Maurer, Sabine Altrichter, Oliver Schmetzer, Jörg Scheffel, Martin K Church, Martin Metz
The study of autoimmunity mediated by immunoglobulin E (IgE) autoantibodies, which may be termed autoallergy, is in its infancy. It is now recognized that systemic lupus erythematosus, bullous pemphigoid (BP), and chronic urticaria, both spontaneous and inducible, are most likely to be mediated, at least in part, by IgE autoantibodies. The situation in other conditions, such as autoimmune uveitis, rheumatoid arthritis, hyperthyroid Graves' disease, autoimmune pancreatitis, and even asthma, is far less clear but evidence for autoallergy is accumulating...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29682739/bullous-pemphigoid-induced-by-dipeptidyl-peptidase-4-inhibitors-eight-cases-with-clinical-and-immunological-characterization
#13
Irene García-Díez, Marta Ivars-Lleó, Daniel López-Aventín, Norito Ishii, Takashi Hashimoto, Pilar Iranzo, Ramon M Pujol, Agustín España, Josep E Herrero-Gonzalez
BACKGROUND: Dipeptidyl peptidase-4 (DPP-4) inhibitors have increasingly been identified as causative agents of bullous pemphigoid. The clinical and immunological characteristics of this pemphigoid variant are still unclear. The objective of our study was to analyze the clinical and immunological features of patients with pemphigoid induced by DPP-4 inhibitors. METHODS: All patients diagnosed with DPP-4 inhibitor-associated bullous pemphigoid at dermatology departments in three Spanish centers during the period 2013 to 2015 were included...
April 23, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29681159/bullous-pemphigoid-a-10-year-study-of-discordant-results-on-direct-immunofluorescence
#14
Jessica G Fudge, Richard I Crawford
BACKGROUND: Bullous pemphigoid (BP) is the most common subepidermal autoimmune disorder characterized by tense bullae. It is associated with circulating autoantibodies against BP antigen-1 and BP antigen-2. Diagnosis is based upon clinical, histopathologic, and immunopathologic examination. Direct immunofluorescence (DIF) of perilesional skin highlights C3 with or without IgG in a linear pattern along the basement membrane. OBJECTIVES: We hypothesized that repeat biopsies may be required for a definitive DIF diagnosis of BP, as initial DIF evaluation may result in a false-negative result...
April 1, 2018: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/29678394/-dermatologic-toxicities-of-immune-checkpoint-inhibitors
#15
REVIEW
V Sibaud, S Boulinguez, C Pagès, L Riffaud, L Lamant, C Chira, S Boyrie, E Vigarios, E Tournier, N Meyer
The development of immune checkpoint inhibitors (monoclonal antibodies targeting PD-1/PD-L1 or CTLA-4) represents a significant advance in the treatment of multiple cancers. Given their particular mechanism of action, which involves triggering CD4+/CD8+ T-cell activation and proliferation, they are associated with a specific safety profile. Their adverse events are primarily immune-related, and can affect practically all organs. In this context, dermatological toxicity is the most common, though it mostly remains mild to moderate and does not require discontinuation of treatment...
April 17, 2018: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/29663327/peripheral-eosinophilia-in-bullous-pemphigoid-prevalence-and-influence-on-the-clinical-manifestation
#16
K Kridin
BACKGROUND: Peripheral eosinophilia has been reported in 50-60% of bullous pemphigoid (BP) patients and correlated positively with disease severity. The association of peripheral eosinophilia with the different morphological characteristics of BP and the presence of tissue eosinophilia has not been established. METHODS: The study was designed as a case-control study. The diagnosis of BP was grounded on the well-established immunopathological criteria. Five age-, sex-, and ethnicity-matched control subjects were randomly selected for each BP patient...
April 16, 2018: British Journal of Dermatology
https://www.readbyqxmd.com/read/29662486/mucosal-involvement-in-bullous-pemphigoid-is-mostly-associated-with-disease-severity-and-to-absence-of-anti-bp230-autoantibody
#17
Ariane Clapé, Céline Muller, Grégory Gatouillat, Sébastien Le Jan, Coralie Barbe, Bach-Nga Pham, Frank Antonicelli, Philippe Bernard
Bullous pemphigoid (BP) is the most common autoimmune bullous disease and typically affects the elderly. Binding of specific autoantibodies to BP180/230 hemidesmosomal components induces an inflammatory response leading to skin blister formation. Unusual manifestations of BP include additional mucous membrane involvement, without pathophysiological knowledge associated to the formation of these lesions. We here performed a prospective study on series of consecutive BP patients with ( n  = 77) and without ( n  = 18) mucosal involvements at baseline to further investigate why some BP patients display mucosal lesion and other not...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29627775/acute-mucocutaneous-methotrexate-toxicity-with-marked-tissue-eosinophilia
#18
Luis J Borda, Andrew Ross, Gabriel Villada, Clara Milikowski
Methotrexate toxicity in mucocutaneous areas is usually not associated with tissue eosinophilia. We describe a case of acute methotrexate-induced mucocutaneous erosions with interface dermatitis and eosinophils. A 76-year-old African-American woman with a history of bullous pemphigoid on methotrexate therapy presented with lower extremity cellulitis, developing oral and cutaneous erosions during hospitalization after daily dosage of methotrexate. Shallow circular cutaneous erosions were found on chest, abdomen and limbs...
April 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29626572/th1-th17-cell-recognition-of-desmoglein-3-and-bullous-pemphigoid-antigen-180-in-lichen-planus
#19
Thomas Schmidt, Farzan Solimani, Robert Pollmann, Ronja Stein, Ansgar Schmidt, Inna Stulberg, Katja Kühn, Rüdiger Eming, Verena Eubel, Peter Kind, Nicole Arweiler, Cassian Sitaru, Michael Hertl
We identified Th1/Th17 cell responses against desmoglein 3 and bullous pemphigoid antigen 180 in lichen planus. In contrast, patients with pemphigus vulgaris and bullous pemphigoid showed significantly higher Th2 cell responses against these autoantigens.
April 4, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29624655/novel-use-of-combination-therapeutic-plasma-exchange-and-rituximab-in-the-treatment-of-nivolumab-induced-bullous-pemphigoid
#20
Alyson V Ridpath, Polina V Rzepka, Sabrina M Shearer, Scott R Scrape, Thomas E Olencki, Benjamin H Kaffenberger
No abstract text is available yet for this article.
April 6, 2018: International Journal of Dermatology
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