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    4823 results match your criteria Bullous Pemphigoid

    1 OF 97

    Genome-wide association study identifies GALC as susceptibility gene for mucous membrane pemphigoid.
    Exp Dermatol 2017 Nov 14. Epub 2017 Nov 14.
    Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
    Background: Mucous membrane pemphigoid (MMP) is a rare, chronic, and often aggressive subepidermal autoimmune blistering disease potentially affecting several mucous membranes with blisters and secondary erosions and scars. The pathogenesis of MMP is poorly understood, and the contribution of genetic predispositions, other than HLA class II allele variants to MMP, is unknown.

    Objectives: The objective of this study is to identify susceptibility genes for MMP in a British cohort of MMP patients. Read More

    Light Chain Deposition Disease with Bullous Skin Lesions Mimicking Atypical Bullous Pemphigoid.
    Acta Derm Venereol 2017 Aug 23. Epub 2017 Aug 23.
    Department of Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux, INSERM UMRS_1165, Université Paris Diderot, 1 avenue Claude Vellefaux, FR-75010 Paris, France.

    Bullous Diseases.
    Curr Probl Dermatol 2018 7;53:64-69. Epub 2017 Nov 7.
    Autoimmune bullous diseases are a heterogeneous group of blistering diseases affecting the skin and/or mucous membrane. Systemic corticosteroids, which are often associated with immunosuppressants, are the main treatment option for these diseases. The 2 main biologics used in the treatment of autoimmune bullous diseases are rituximab, especially in pemphigus and mucous membrane pemphigoid, and omalizumab in bullous pemphigoid. Read More

    Acute Flare of Bullous Pemphigus With Pembrolizumab Used for Treatment of Metastatic Urothelial Cancer.
    J Immunother 2017 Nov 3. Epub 2017 Nov 3.
    Departments of *Hematology and Oncology †Internal Medicine ‡Dermatology, University of Iowa Hospitals and Clinics, Iowa City, IA.
    In the past decade, the resurgence of immunotherapy has changed the landscape of cancer therapy. Checkpoint inhibitors targeting cytotoxic T-lymphocyte antigen-4, programmed death-1 on lymphocytes, and programmed death ligand-1 on tumors cells are currently utilized in the management of several cancers. These agents are double-edged sword with the positive effect being robust antitumor response but on the other side they can throttle up the normal immunologic homeostasis in a negative way, leading to adverse autoimmune toxicities. Read More

    Biomarkers related to bullous pemphigoid activity and outcome.
    Exp Dermatol 2017 Nov 3. Epub 2017 Nov 3.
    Laboratory of Dermatology, Faculty of Medicine of Reims, EA 7319, IFR CAP Santé, University of Reims Champagne-ArdenneReims, France.
    Bullous pemphigoid (BP) is the most common autoimmune sub-epidermal blistering disease of the skin. Investigation of the BP-associated pathophysiological processes during the last decades showed that the generation of auto-antibodies directed against the hemidesmosome proteins BP180 and BP230, a hallmark of the BP-associated autoimmune response, leads to the recruitment of inflammatory immune cells at the dermal-epidermal junction, and subsequently to the release of a large amount of inflammatory molecules involved in blister formation. Analysis in transversal and longitudinal studies of autoantibodies and inflammatory molecules production both at time of diagnosis and under treatment was mainly performed within the serum but also in the blister fluid. Read More

    Nonbullous cutaneous pemphigoid: a systematic review.
    J Am Acad Dermatol 2017 Nov 1. Epub 2017 Nov 1.
    University of Groningen, University Medical Center Groningen, Department of Dermatology, Center for Blistering Diseases, Groningen, the Netherlands. Electronic address:
    Background: Cutaneous pemphigoid (bullous pemphigoid) is an autoimmune bullous disease that typically presents with tense bullae and severe pruritus. However, bullae may be lacking, a subtype termed nonbullous cutaneous pemphigoid.

    Objective: To summarize the reported characteristics of nonbullous cutaneous pemphigoid. Read More

    Ethnic variations in the epidemiology of bullous pemphigoid in Israel.
    Int J Dermatol 2017 Oct 31. Epub 2017 Oct 31.
    Department of Dermatology, Rambam Health Care Campus, Haifa, Israel.
    Background: No ethnic or geographic predisposition to bullous pemphigoid (BP) was reported so far.

    Objective: To evaluate trends in the incidence of BP in northern Israel, shedding light on differences between two distinct ethnic populations who inhabit the same region, namely Jews and Arabs.

    Methods: Bullous pemphigoid incidence was retrospectively estimated from January 2000-December 2015 in two Israeli regions with a total population of 1. Read More

    IgE autoantibodies and their association with the disease activity and phenotype in bullous pemphigoid: a systematic review.
    Arch Dermatol Res 2017 Oct 25. Epub 2017 Oct 25.
    School of Life Sciences, University of Nottingham, Life Sciences Building, University Park, Nottingham, NG7 2RD, UK.
    Bullous pemphigoid (BP) is the most common autoimmune skin disease of blistering character. The underlying pathophysiological mechanism involves an immune attack, usually by IgG class autoantibodies, on the autoantigen BP 180/BPAg2, which is a type XVII collagen (COL17) protein acting as the adhesion molecule between the epidermis and the basement membrane of the dermis. About 40 years ago, following consistent findings of elevated total serum IgE levels in BP patients, it was hypothesized that IgE may be involved in the pathophysiology of BP. Read More

    Semaphorin 4D from CD15(+) Granulocytes via ADAM10-induced Cleavage Contributes to Antibody Production in Bullous Pemphigoid.
    J Invest Dermatol 2017 Oct 17. Epub 2017 Oct 17.
    Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi, China. Electronic address:
    Autoreactive B cells activation and antibody production are critical events for the development of bullous pemphigoid (BP). However, the mechanism that is involved in the modulation of B cell activation and autoantibody generation has not been fully understood. Semaphorin 4D (Sema4D/CD100) plays important roles in immune regulation related to B cell, but its implication in BP remains obscure. Read More

    Spectrum of orocutaneous disease associations: Immune-mediated conditions.
    J Am Acad Dermatol 2017 Nov;77(5):795-806
    Division of Dermatology, Baylor University Medical Center, Dallas, Texas. Electronic address:
    There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies. Read More

    Lupus-like cutaneous reaction following pembrolizumab: An immune-related adverse event associated with anti-PD-1 therapy.
    J Cutan Pathol 2017 Oct 13. Epub 2017 Oct 13.
    Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
    PD-1 (programmed cell death-1) inhibitors, used to treat metastatic melanoma and other malignancies, are associated with development of immune-related adverse events in the skin. Such reactions include morbilliform eruptions, vitiligo, alopecia areata and bullous pemphigoid. In this report, we describe a patient who developed a lupus-like cutaneous reaction in the setting of pembrolizumab therapy for metastatic melanoma, adding to the spectrum of reactions which may be observed in association with PD-1 inhibitor therapy. Read More

    A comparative study of expression of Fc receptors in relation to the autoantibody-mediated immune response and neutrophil elastase expression in autoimmune blistering dermatoses.
    Pol J Pathol 2017;68(2):109-116
    Here we investigated the cutaneous CD32A and CD89 expression in relation to the neutrophil elastase (NE) expression and serum level of anti-desmoglein 1 and 3 (DSG1/DSG3) IgG in pemphigus, anti-BP180/BP230 IgG in bullous pemphigoid (BP), anti-gliadin nonapeptides (npG), tissue (tTG), and epidermal transglutaminases (eTG) IgA in dermatitis herpetiformis (DH). The examined material consisted of skin/mucosal tissues and sera. In total, 87 patients were studied. Read More


    Man with leg rash.
    Emerg Med J 2017 Oct;34(10):686-691
    Department of Emergency Medicine, Harbor-UCLA Medical Center, Torrance, USA.
    Clinical Introduction: A 51-year-old male presented to the ED with a rash to his left thigh (figure 1) with erythema, swelling and pain. He endorsed paraesthesias, pruritus, fevers, vomiting and diarrhoea. Initial vitals were unremarkable. Read More

    Doxycycline compared to prednisolone therapy for patients with bullous pemphigoid: cost-effectiveness analysis of the BLISTER trial.
    Br J Dermatol 2017 Sep 22. Epub 2017 Sep 22.
    Centre of Evidence Based Dermatology, University of Nottingham, NG7 2NR, UK.
    Background: Bullous pemphigoid (BP) is an autoimmune blistering skin disorder associated with significant morbidity and mortality. Doxycycline and prednisolone to treat bullous pemphigoid were compared within a randomised controlled trial (RCT).

    Objectives: To compare the cost-effectiveness of doxycycline-initiated and prednisolone-initiated treatment for patients with BP. Read More

    Structural proteins of the dermal-epidermal junction targeted by autoantibodies in pemphigoid diseases.
    Exp Dermatol 2017 Sep 8. Epub 2017 Sep 8.
    Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.
    The dermal-epidermal junction consists of a network of several interacting structural proteins which strengthen adhesion and mediate signaling events. This structural network consists of hemidesmosomal-anchoring filament complexes connecting the basal keratinocytes to the basement membrane. The anchoring filaments in turn interact with the anchoring fibrils to attach the basement membrane to the underlying dermis. Read More

    Variation of the epidermal expression of glucocorticoid receptor-beta as potential predictive marker of bullous pemphigoid outcome.
    Exp Dermatol 2017 Sep 8. Epub 2017 Sep 8.
    Laboratory of Dermatology, Faculty of Medicine of Reims, EA 7319, IFR CAP Santé, University of Reims Champagne-Ardenne, Reims, France.
    Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries. Although topical and/or systemic glucocorticoids treatment efficacy is widely recognized, up to 30% of patients with BP may undergo a relapse during the first year of treatment. We investigated the protein expression of the total glucocorticoid receptor and GRβ isoform in the skin biopsy specimens from patients with BP and wondered whether such investigation at baseline provided a tool to predict disease outcome. Read More

    Scabies masquerading as bullous pemphigoid: scabies surrepticius.
    Clin Cosmet Investig Dermatol 2017 23;10:317-324. Epub 2017 Aug 23.
    Department of Dermatology, University of California San Diego, La Jolla, CA, USA.
    Scabies, a parasitic infestation caused by the mite Sarcoptes scabiei, is diagnosed by observing either the mite, its ova, or its excrement. The mite tracts, known as burrows and a characteristic presentation of the pruritic condition, are typically found on the web spaces between the fingers. Other cutaneous lesions include excoriated papules, pustules, and vesicles. Read More

    Incidence of bullous pemphigoid in Sweden 2005-2012: a nationwide population-based cohort study of 3761 patients.
    Arch Dermatol Res 2017 Nov 5;309(9):721-727. Epub 2017 Sep 5.
    Department of Dermatology, Skåne University Hospital, Lund University, Malmö, Sweden.
    Studies that report the incidence of bullous pemphigoid from validated nationwide population-based registries are rare. The aim of this study was to estimate the incidence of bullous pemphigoid in Sweden 2005-2012. A population-based open cohort study was designed including all patients diagnosed by a dermatologist with bullous pemphigoid (BP) in Sweden from 2005 to 2012 (n = 3761), identified from the National Patient Register (NPR). Read More

    IgE auto-reactivity in bullous pemphigoid: eosinophils and mast cells as major targets of pathogenic immune reactants.
    Br J Dermatol 2017 Sep 4. Epub 2017 Sep 4.
    Department of Dermatology Division of Immunology, Allergy and Infectious Diseases, Medical University of Vienna, Vienna, Austria.
    Background: Bullous pemphigoid (BP) is an autoimmune disease characterised by tense blisters that are usually preceded by urticarial eruptions. Affected patients exhibit IgG and/or IgE auto-antibodies against BP180 and/or BP230. Their relative importance in disease pathogenesis has not been fully elucidated. Read More

    Oral mucosa is a useful substrate for detecting autoantibodies of mucous membrane pemphigoid.
    Br J Dermatol 2017 Sep 2. Epub 2017 Sep 2.
    Department of Dermatology, Hokkaido University Graduate School of Medicine.
    Mucous membrane pemphigoid (MMP) is a rare autoimmune blistering disease targeting various autoantigens, including the C-terminus of collagen XVII (COL17) and laminin332. Bullous pemphigoid (BP) is the most common autoimmune blistering disease, affecting the mucosae in 10-20% of cases. COL17, particularly, the non-collagenous (NC)16A domain is the pathogenic epitope for BP. Read More

    Trichoscopy - a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases.
    Int J Dermatol 2017 Oct 30;56(10):996-1002. Epub 2017 Aug 30.
    Department of Dermatology, Medical University of Warsaw, Warsaw, Poland.
    Background: Scalp is a common location of autoimmune bullous diseases. Trichoscopy is a noninvasive method for diagnosing hair and scalp diseases. Data on trichoscopy in autoimmune bullous diseases are limited to the studies on pemphigus including a small number of patients. Read More

    Failure of initial disease control in bullous pemphigoid: a retrospective study of hospitalized patients in a single tertiary center.
    Int J Dermatol 2017 Oct 30;56(10):1010-1016. Epub 2017 Aug 30.
    Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
    Background: Bullous pemphigoid (BP) is the most prevalent autoimmune blistering skin disease in Western countries and in Israel. Initial disease control is achieved in 60-90% of BP patients within 1-4 weeks of corticosteroid therapy. In the remainder of patients, recalcitrant disease is controlled with additional immunosuppressive treatment. Read More

    Determining the Incidence of Pneumocystis Pneumonia in Patients With Autoimmune Blistering Diseases Not Receiving Routine Prophylaxis.
    JAMA Dermatol 2017 Nov;153(11):1137-1141
    Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
    Importance: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. Read More

    Mortality in bullous pemphigoid and prognostic factors in 1st and 3rd year of follow-up in specialized centre in Poland.
    Arch Dermatol Res 2017 Nov 29;309(9):709-719. Epub 2017 Aug 29.
    Department of Dermatology and Immunodermatology, Medical University of Warsaw, Koszykowa 82a Str, 02-008, Warsaw, Poland.
    Bullous pemphigoid (BP) is associated with higher mortality and coexisting comorbidities, some of them affecting poor prognosis. The aim of the study was to identify prognostic factors causing greater mortality both in the 1st and 3rd year of follow-up and to determine the 1-, 2-, 3-year mortality rates, standardized mortality ratio (SMR) in Polish BP patients. All patients with BP (a cohort of 205 patients, mean age 76. Read More

    Eosinophils as putative therapeutic targets in bullous pemphigoid.
    Exp Dermatol 2017 Aug 20. Epub 2017 Aug 20.
    Institute of Pharmacology, University of Bern, Bern, Switzerland.
    Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering skin disease and is characterized by the presence of autoantibodies directed against the hemidesmosomal proteins BP180 and BP230 that can be detected in the skin and serum of BP patients. Histologically, the dermal infiltration of eosinophils is obvious. The objective of this review was to present evidence that eosinophils play a key role in the pathogenesis of BP. Read More

    In vitro and in vivo models to investigate the pathomechanisms and novel treatments for pemphigoid diseases.
    Exp Dermatol 2017 Aug 20. Epub 2017 Aug 20.
    Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
    Pemphigoid diseases (PD) are a subgroup of rare acute or chronic autoimmune skin disorders characterized and caused by autoantibodies directed against distinct structural components of the dermal-epidermal junction. Binding of autoantibodies to their targets leads to the formation of blisters and erosions in patients. PDs comprise eight disorders for which the molecular target antigens have been identified. Read More

    Bullous Pemphigoid Associated with the Dipeptidyl Peptidase-4 Inhibitor Sitagliptin in a Patient with Liver Cirrhosis Complicated with Rapidly Progressive Hepatocellular Carcinoma.
    Intern Med 2017 Sep 21;56(18):2471-2474. Epub 2017 Aug 21.
    Uchiwa Clinic, Japan.
    A 78-year-old man presented with cutaneous blisters of the limbs and abdominal distension. He had been treated for various diseases, including liver cirrhosis. He had begun receiving sitagliptin, a dipeptidyl peptidase-4 (DPP-4) inhibitor, for diabetes mellitus three years before the hospitalization. Read More

    Cutaneous Eruptions in Patients Receiving Immune Checkpoint Blockade: Clinicopathologic Analysis of the Nonlichenoid Histologic Pattern.
    Am J Surg Pathol 2017 Oct;41(10):1381-1389
    Departments of *Dermatology ‡Pathology §Oncology, Johns Hopkins University School of Medicine and Sidney Kimmel Comprehensive Cancer Center ∥The Brady Urologic Institute, Johns Hopkins University School of Medicine, Baltimore, MD Departments of †Medicine, Thoracic Oncology Service ¶Pathology #Dermatology, Memorial Sloan Kettering Cancer Center, New York, NY.
    Cutaneous eruptions are among the most common immune-related adverse events (irAEs) associated with anti-programmed cell death protein 1/programmed cell death ligand 1 therapy, and are often clinically and histologically characterized as lichenoid. Nonlichenoid patterns may also occur and are likely to be encountered by surgical pathologists, given the increasing clinical use of these agents. The purpose of this study is to describe the histopathologic features of nonlichenoid cutaneous irAEs from patients receiving anti-programmed cell death protein 1/programmed cell death ligand 1 therapies for a variety of underlying advanced malignancies. Read More

    The Value of Direct Immunofluorescence on Proteinase-Digested Formalin-Fixed Paraffin-Embedded Skin Biopsies.
    Am J Dermatopathol 2017 Aug 9. Epub 2017 Aug 9.
    Department of Pathology, University of Massachusetts Medical School, UMass Memorial Medical Center, Worcester, MA.
    Direct immunofluorescence (DIF) on frozen tissue (DIF-F) is the method of choice for the identification of immune deposits present in skin and other tissues. DIF can also be performed on formalin-fixed paraffin-embedded tissue (DIF-P) after antigen retrieval with proteases and has proven to be of value in renal pathology. However, its utility in skin biopsies has not been fully examined. Read More

    The Role of Intereukin-31 in Pathogenesis of Itch and Its Intensity in a Course of Bullous Pemphigoid and Dermatitis Herpetiformis.
    Biomed Res Int 2017 20;2017:5965492. Epub 2017 Jul 20.
    Department of Dermatology and Venereology, Medical University of Lodz, Poland Hallera Square No. 1, 90-647 Lodz, Poland.
    Itch which is one of the major, subjective symptoms in a course of bullous pemphigoid and dermatitis herpetiformis makes those two diseases totally different than other autoimmune blistering diseases. Its pathogenesis is still not fully known. The aim of this research was to assess the role of IL-31 in development of itch as well as to measure its intensity. Read More

    Autoimmune Bullous Disease in Childhood.
    Indian J Dermatol 2017 Jul-Aug;62(4):440
    Department of Dermatology, School of Medicine, Marmara University, Istanbul, Turkey.
    Background: Autoimmune bullous disorders (AIBDs) are a heterogeneous group of diseases which are rarely seen in children. Studies concerning the immunobullous diseases in pediatric patients are scarce.

    Aims And Objectives: In this study, we aimed to investigate the clinical features and treatment outcomes of AIBDs in children. Read More

    Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.
    Clin Rev Allergy Immunol 2017 Aug 4. Epub 2017 Aug 4.
    Department of Dermatology, University of Bern, Bern, Switzerland.
    Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. Read More

    Glucocorticoids: The mode of action in bullous pemphigoid.
    Exp Dermatol 2017 Aug 3. Epub 2017 Aug 3.
    PEDEGO Research Unit, Oulu Center for Cell-Matrix Research, Department of Dermatology and Medical Research Center Oulu, Oulu University Hospital and University of Oulu, Oulu, Finland.
    Bullous pemphigoid (BP) is the most common of pemphigoid diseases caused by autoantibodies against the structures of dermoepidermal junction followed by complement activation, innate immune cell infiltration, neutrophil proteinase secretion and subepidermal blister formation. The first-line treatment of BP is topical and systemic glucocorticoids (GC). Regulation of the immune system and inflammatory cells is the main target of GC actions. Read More

    Skin diseases of the vulva: inflammatory, erosive-ulcerating and apocrine gland diseases, zinc and vitamin deficiency, vulvodynia and vestibulodynia.
    J Obstet Gynaecol 2017 Aug 1:1-12. Epub 2017 Aug 1.
    a Department of Dermatology , Bispebjerg Hospital , Copenhagen , Denmark.
    Chronic, inflammatory and ulcerating mucocutaneous diseases that can affect the vulvar area are reviewed: lichen sclerosus, lichen planus, plasma cell vulvitis, complex aphthosis, Behcet's disease, pyoderma gangrenosum, metastatic Crohn's disease, dyskeratotic skin diseases (Hailey-Hailey disease and Darier's disease), autoimmune bullous diseases (mucous membrane pemphigoid and pemphigus vulgaris) and hidradenitis suppurativa. Also, vulvodynia and vestibulodynia, zinc and vitamin B deficiency are described. Read More

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