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    855 results match your criteria Epidermolysis Bullosa Acquisita

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    International Bullous Diseases Group - Consensus on Diagnostic Criteria for Epidermolysis Bullosa Acquisita.
    Br J Dermatol 2017 Nov 22. Epub 2017 Nov 22.
    Department of Dermatology at St George Hospital, University of New South Wales, Sydney, Australia.
    Background: Epidermolysis bullosa acquisita (EBA) is a complex autoimmune bullous disease disease with variable clinical presentations and multiple possible diagnostic tests making an international consensus on diagnosis of EBA needed.

    Objectives: To obtain an international consensus on the clinical and diagnostic criteria for EBA.

    Methods: The international bullous diseases group (IBDG) met three times to discuss the clinical and diagnostic criteria for EBA. Read More

    New insights into pemphigoid diseases.
    Exp Dermatol 2017 Nov 21. Epub 2017 Nov 21.
    Lübeck Institute of Experimental Dermatology, University of Lübeck, Germany.
    Pemphigus and pemphigoid diseases are organ-specific autoimmune blistering diseases (AIBD), characterized and caused by autoantibodies to structural components of the skin (1). The autoantigens targeted in pemphigus are desmoglein 1 and 3, two proteins of the desmosomal structure, while the autoantigens in pemphigoid diseases (PD) are components of the basal membrane. For example, bullous pemphigoid (BP), the most frequent PD is characterized by autoantibodies against type XVII collagen (COL17, BP180) and BP230, and epidermolysis bullosa acquisita (EBA) is caused by autoantibodies against type VII collagen (COL7). Read More

    Subepidermal autoimmune bullous diseases: overview, epidemiology, and associations.
    Immunol Res 2017 Nov 21. Epub 2017 Nov 21.
    Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel.
    Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different regions of the world. Read More

    Serration pattern analysis for differentiating epidermolysis bullosa acquisita from other pemphigoid diseases.
    J Am Acad Dermatol 2017 Nov 15. Epub 2017 Nov 15.
    University of Groningen, University Medical Center Groningen, Department of Dermatology, Center for Blistering Diseases, Groningen, the Netherlands. Electronic address:
    Background: Direct immunofluorescence microscopy (DIF) of a skin biopsy specimen is the reference standard for the diagnosis of pemphigoid diseases (PD). Serration pattern analysis enables differentiation of epidermolysis bullosa acquisita (EBA) from other PD using DIF microscopy alone. However, practice gaps need to be addressed for implication of this technique in daily routine diagnostics. Read More

    Efficacy of intravenous immunoglobulins for the treatment of mucous membrane pemphigoid-like epidermolysis bullosa acquisita.
    Eur J Dermatol 2017 Oct;27(5):563-564
    Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan, Singapore Immunology Network (SIgN) and Institute of Medical Biology, Agency for Science, Technology and Research (A*STAR), Biopolis, Singapore.

    Response to 'Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita - a multicenter analysis'.
    Br J Dermatol 2017 Oct 4. Epub 2017 Oct 4.
    University of Groningen, University Medical Center Groningen, Department of Dermatology, Center for Blistering Diseases, Groningen, the Netherlands.
    In a recent retrospective serological study in 95 sera from epidermolysis bullosa acquisita (EBA) patients Schmidt et al. conclude that Col7A-NC1/NC2 ELISA (MBL, Japan) is superior to NC1 ELISA, Western blot and indirect immunofluorescence (IIF) on salt-split skin (SSS) with the highest sensitivity of 97.9%. Read More

    Calcitriol treatment ameliorates inflammation and blistering in mouse models of epidermolysis bullosa acquisita.
    J Invest Dermatol 2017 Sep 20. Epub 2017 Sep 20.
    Department of Dermatology, University of Lübeck, Germany.
    A link between hypovitaminosis D and development of autoimmune bullous disorders has been recently suggested, but this association has not been experimentally elaborated. Here, the role of vitamin D was investigated in epidermolysis bullosa acquisita (EBA), an anti-type VII collagen autoantibody-induced blistering skin disease. Oral administration of the hormonally active vitamin D metabolite calcitriol ameliorated clinical disease severity and dermal neutrophil infiltration in both an antibody transfer- and immunization-induced EBA mouse model. Read More

    Nanoparticles prepared from porcine cells support the healing of cutaneous inflammation in mice and wound re-epithelialization in human skin.
    Exp Dermatol 2017 Sep 23. Epub 2017 Sep 23.
    Institute of Anatomy, University of Luebeck, Luebeck, Germany.
    Previous reports have demonstrated that cell-derived nanoparticles (CDNPs) composed of bovine or porcine protein complexes exerted therapeutic effects against viral infections and cancer in mice and humans. Based on these observations, we asked whether CDNPs would improve inflammatory skin disorders. To address this, we utilized two distinct mouse models of cutaneous inflammation: the autoimmune skin-blistering disease epidermolysis bullosa acquisita (EBA) as an example of an autoantibody-induced cutaneous inflammation, and Leishmania major (L. Read More

    Evidence for a contributory role of a xenogeneic immune response in experimental epidermolysis bullosa acquisita.
    Exp Dermatol 2017 Sep 8. Epub 2017 Sep 8.
    Institute of Anatomy, University of Lübeck, Germany.
    Autoimmune diseases affect a large fraction of the population in Western countries. To elucidate the underlying causes, autoantibody transfer-induced mouse models have been established that greatly contributed to the understanding of the pathophysiology of these diseases. However, the role of a potentially co-occurring murine xenogeneic immune response to commonly utilized rabbit anti-mouse IgG remains poorly understood. 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

    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

    Colchicine: an ancient drug with novel applications.
    Br J Dermatol 2017 Aug 18. Epub 2017 Aug 18.
    Division of Basic and Translational Biophysics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, NIH, Bethesda, MD.
    Colchicine is a historic treatment for gout that has been used for more than a millennium. It is the treatment of choice for Familial Mediterranean Fever and its associated complication, amyloidosis. The 2009 FDA approval of colchicine as a new drug had research consequences. 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

    Epidermolysis bullosa acquisita and anti-p200 pemphigoid as major subepidermal autoimmune bullous diseases diagnosed by floor binding on indirect immunofluorescence microscopy using human salt-split skin.
    Indian J Dermatol Venereol Leprol 2017 Sep-Oct;83(5):550-555
    Department of Dermatology, University of Lübeck, Lübeck, Germany.
    Background: Subepidermal autoimmune bullous diseases are a diverse group of diseases with overlapping clinical and immunopathological features. Indirect immunofluorescence microscopy on artificially split skin helps to classify these conditions into those with staining on the epidermal side of the split ("roof-binding") and those with staining on the dermal side ("floor-binding"). Epidermolysis bullosa acquisita is the prototype of "floor-binding" subepidermal autoimmune bullous diseases. Read More

    Dimethyl fumarate modulates neutrophil extracellular trap formation in a glutathione and superoxide-dependent manner.
    Br J Dermatol 2017 Jul 22. Epub 2017 Jul 22.
    Department of Dermatology, University of Heidelberg, Heidelberg, Germany.
    Background: Neutrophil (polymorphonuclear) granulocytes (PMN) were shown to contribute to the pathogenesis of psoriasis by releasing IL-17 and LL-37/ DNA complexes via neutrophil extracellular traps (NET), webs of chromatin strands decorated with antimicrobial peptides, in psoriatic skin. Fumaderm(®) , a fumaric acid ester (FAE) formulation consisting of different FAE salts has been successfully used to treat psoriasis for decades. Most recently, FAE treatment was reported to inhibit NET formation in murine epidermolysis bullosa acquisita. Read More

    Distinguishing Epidermolysis Bullosa Acquisita From Bullous Pemphigoid Without Direct Immunofluorescence.
    J Cutan Med Surg 2017 Jul 1:1203475417722734. Epub 2017 Jul 1.
    1 Departments of Dermatology and Skin Science, University of British Columbia, Vancouver, BC, Canada.
    Background: It has been postulated that periodic acid-Schiff staining of basement membrane can predict direct immunofluorescence patterns seen in epidermolysis bullosa acquisita and bullous pemphigoid. It has also been suggested that the type of inflammatory infiltrate or presence of fraying of basal keratinocytes may differentiate these two conditions.

    Objective: In this study, we aimed to confirm these observations. Read More

    CCL3/MIP1α represents a biomarker but not a mandatory cytokine for disease development in experimental epidermolysis bullosa acquisita.
    J Dermatol Sci 2017 Nov 30;88(2):248-250. Epub 2017 Jun 30.
    Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

    Evaluation of Autoimmune Bullous Diseases in Elderly Patients in Iran: A 10-Year Retrospective Study.
    Skinmed 2017 1;15(3):175-180. Epub 2017 Jun 1.
    Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Read More

    Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita - a multicenter analysis.
    Br J Dermatol 2017 Jul 13. Epub 2017 Jul 13.
    Department of Dermatology and Allergology, Philipps-University, Marburg, Germany.
    Background: Epidermolysis bullosa acquisita is a rare, potentially devastating autoimmune disease of the skin. IgG autoantibodies directed against type VII collagen (Col7), the major component of anchoring fibrils, induce skin fragility leading to cutaneous and mucocutaneous blister formation which is mostly of a scaring phenotype. Thus, powerful and reproducible diagnostic assays are critical to establish the diagnosis of EBA early to avoid irreversible sequelae. Read More

    A rare occurrence of epidermolysis bullosa acquisita in a patient with retroviral disease.
    Int J STD AIDS 2017 Oct 1;28(12):1255-1258. Epub 2017 Mar 1.
    Department of Dermatology, Topiwala National Medical College & B.Y.L. Nair Ch. Hospital, Mumbai, India.
    Epidermolysis bullosa acquisita is a chronic subepidermal blistering disease associated with autoimmunity to type-VII collagen within anchoring fibrils located at the dermo-epidermal junction. This entity is rarely reported from India. It can have a variety of presentations. Read More

    The Syk Tyrosine Kinase Is Required for Skin Inflammation in an In Vivo Mouse Model of Epidermolysis Bullosa Acquisita.
    J Invest Dermatol 2017 Oct 30;137(10):2131-2139. Epub 2017 May 30.
    Department of Physiology, Semmelweis University School of Medicine, Budapest, Hungary; MTA-SE "Lendület" Inflammation Physiology Research Group of the Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary. Electronic address:
    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. Read More

    Epidermolysis Bullosa Acquisita (Brunsting-Perry Pemphigoid Variant) Localized to the Face and Diagnosed With Antigen Identification Using Skin Deficient in Type VII Collagen.
    Am J Dermatopathol 2017 Jul;39(7):e90-e96
    Departments of *Dermatology, and †Cellular Pathology, St George's University Hospital, London, United Kingdom; and ‡Department of Immunodermatology, St Thomas' Hospital, London, United Kingdom.
    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. Read More

    Human orf complicated by epidermolysis bullosa acquisita.
    Br J Dermatol 2017 Mar 24. Epub 2017 Mar 24.
    Dermatology Department, Saint-Louis Hospital, 1 Avenue Claude Vellefaux, 75010, Paris, France.
    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 autoimmune bullous dermatosis complicating orf disease have been reported to date. Read More

    Signalling and targeted therapy of inflammatory cells in epidermolysis bullosa acquisita.
    Exp Dermatol 2017 Mar 7. Epub 2017 Mar 7.
    Lübeck Institute of Experimental Dermatology and Department of Dermatology, University of Lübeck, Lübeck, Germany.
    Pemphigoid diseases (PDs) are chronic and life-threatening autoimmune diseases of the skin and mucous membranes. PDs are characterized and caused by autoantibodies targeting components of the basement membrane. In the PD epidermolysis bullosa acquisita (EBA), the target autoantigen is type VII collagen. Read More

    Unique mouse monoclonal antibodies reactive with maturation-related epitopes on type VII collagen.
    Exp Dermatol 2017 Sep 20;26(9):811-819. Epub 2017 Apr 20.
    Department of Dermatology, Kurume University School of Medicine, Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.
    In this study, we generated a new set of monoclonal antibodies (mAbs) to bovine and human type VII collagen (COL7) by immunizing mice with bovine cornea-derived basement membrane zone (BMZ) fraction. The four mAbs, tentatively named as COL7-like mAbs, showed speckled subepidermal staining in addition to linear BMZ staining of normal human skin and bovine cornea, a characteristic immunofluorescence feature of COL7, but showed no reactivity with COL7 by in vitro biochemical analyses. Taking advantage of the phenomenon that COL7-like mAbs did not react with mouse BMZ, we compared immunofluorescence reactivity between wild-type and COL7-rescued humanized mice and found that COL7-like mAbs reacted with BMZ of COL7-rescued humanized mice. Read More

    The Leukotriene B4 and its Receptor BLT1 Act as Critical Drivers of Neutrophil Recruitment in Murine Bullous Pemphigoid-Like Epidermolysis Bullosa Acquisita.
    J Invest Dermatol 2017 May 17;137(5):1104-1113. Epub 2017 Jan 17.
    Department of Dermatology, Allergy, and Venereology, University of Lübeck, 23538 Lübeck, Germany. Electronic address:
    Recruitment of neutrophils and eosinophils into the skin is a hallmark of pemphigoid diseases. The molecular cues regulating granulocyte recruitment into the skin and the individual contributions of neutrophils and eosinophils to pemphigoid diseases are, however, poorly understood. The lipid mediator leukotriene B4 (LTB4) is a potent granulocyte chemoattractant and is abundant in the skin blister fluid of bullous pemphigoid (BP) patients, but its pathogenic significance is unknown. Read More

    Bullous, pseudobullous, & pustular dermatoses.
    Semin Diagn Pathol 2017 May 14;34(3):250-260. Epub 2016 Dec 14.
    Section of Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, United States. Electronic address:
    Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Read More

    Prospective studies on the routine use of a novel multivariant enzyme-linked immunosorbent assay for the diagnosis of autoimmune bullous diseases.
    J Am Acad Dermatol 2017 May 28;76(5):889-894.e5. Epub 2016 Dec 28.
    Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany. Electronic address:
    Background: Serologic diagnosis of autoimmune blistering disease (AIBD) usually follows a sophisticated multistep algorithm.

    Objective: We sought validation of a multivariant enzyme-linked immunosorbent assay (ELISA) in the routine diagnosis of AIBD.

    Methods: The multivariant ELISA comprising 6 recombinant immunodominant forms of major AIBD target antigens, ie, desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, and type VII collagen was applied in: (1) a cohort of well-characterized AIBD (n = 173) and control sera (n = 130), (2) a prospective multicenter study with 204 sera from patients with newly diagnosed AIBD with positive direct immunofluorescence microscopy, and (3) a prospective monocenter study with 292 consecutive sera from patients with clinical suspicion of AIBD in comparison with the conventional multistep diagnostic algorithm. Read More

    Research Techniques Made Simple: Mouse Models of Autoimmune Blistering Diseases.
    J Invest Dermatol 2017 Jan;137(1):e1-e6
    Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany. Electronic address:
    Autoimmune blistering diseases are examples of autoantibody-mediated, organ-specific autoimmune disorders. Based on a genetic susceptibility, such as a strong HLA-class II association, as yet unknown triggering factors induce the formation of circulating and tissue-bound autoantibodies that are mainly directed against adhesion structures of the skin and mucous membranes. Compared with other autoimmune diseases, especially systemic disorders, the pathogenicity of autoimmune blistering diseases is relatively well described. Read More

    Reduced skin blistering in experimental epidermolysis bullosa acquisita after anti-TNF treatment.
    Mol Med 2016 Dec 20;23. Epub 2016 Dec 20.
    Lübeck Institute of Experimental Dermatology, University of Lübeck, Germany.
    Epidermolysis bullosa acquisita (EBA) is a difficult-to-treat subepidermal autoimmune blistering skin disease (AIBD) with circulating and tissue-bound anti-type VII collagen antibodies. Different reports have indicated an increased concentration of tumor necrosis factor alpha (TNF) in the serum and blister fluid of patients with subepidermal AIBDs. Furthermore, successful anti-TNF treatment has been reported for individual patients with AIBDs. Read More

    Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus.
    Case Rep Dermatol 2016 Sep-Dec;8(3):278-282. Epub 2016 Oct 13.
    Department of Internal Medicine, Advocate Illinois Masonic Medical Center, Chicago, Ill., USA.
    Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Read More

    T cells mediate autoantibody-induced cutaneous inflammation and blistering in epidermolysis bullosa acquisita.
    Sci Rep 2016 Dec 5;6:38357. Epub 2016 Dec 5.
    Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Ratzeburger Allee 160, D-23538 Lübeck, Germany.
    T cells are key players in autoimmune diseases by supporting the production of autoantibodies. However, their contribution to the effector phase of antibody-mediated autoimmune dermatoses, i.e. Read More

    PDE4 Inhibition as Potential Treatment of Epidermolysis Bullosa Acquisita.
    J Invest Dermatol 2016 Nov 5;136(11):2211-2220. Epub 2016 Jul 5.
    Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany; Department of Dermatology, University of Lübeck, Lübeck, Germany.
    Pemphigoid diseases such as epidermolysis bullosa acquisita (EBA) may be difficult to treat. In pemphigoid diseases, mucocutaneous blistering is caused by autoantibodies to hemidesmosomal antigens; in EBA the autoantigen is type VII collagen. Despite growing insights into pemphigoid disease pathogenesis, corticosteroids are still a mainstay of treatment. Read More

    Autoimmune Blistering Diseases in the Elderly: Clinical Presentations and Management.
    Drugs Aging 2016 Oct;33(10):711-723
    Department of Dermatology, St. George Hospital, Gray St, Kogarah, Sydney, 2217, NSW, Australia.
    Elderly patients are more susceptible to the development of autoimmune blistering disorders such as bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and paraneoplastic pemphigus. This article focuses on the clinical aspects of the aforementioned autoimmune blistering diseases and highlights the important factors involved in treating elderly patients. It is essential for clinicians to offer individualized treatment plans for these patients to optimize outcomes, as elderly patients often have multiple co-morbidities, polypharmacy, and suboptimal socioeconomic status that can adversely influence adequate compliance. Read More

    Incidence of autoimmune bullous diseases in Serbia: a 20-year retrospective study.
    J Dtsch Dermatol Ges 2016 Oct;14(10):995-1005
    Institute of Social Medicine, Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
    Background And Objectives: While most previous surveys on the clinico-epidemiological features of autoimmune bullous diseases (AIBDs) have predominantly focused on a single disease entity or just one disease group, there have been only few studies examining the incidence of various AIBDs. In the present study, we set out to determine the spectrum of AIBDs, to estimate the incidence of the most common AIBDs, and to examine their temporal trends in Central Serbia over a period of 20 years.

    Methods: We retrospectively recruited 1,161 new AIBD cases diagnosed in Central Serbia during the period from January 1991 to December 2010. Read More

    Inzidenz von bullösen Autoimmunerkrankungen in Serbien: eine retrospektive Studie über 20 Jahre.
    J Dtsch Dermatol Ges 2016 Oct;14(10):995-1006
    Institut für Sozialmedizin, Medizinische Fakultät, Universität Belgrad, Belgrad, Serbien.
    Hintergrund Und Ziele: Die meisten früheren Arbeiten zu den klinisch-epidemiologischen Merkmalen von bullösen Autoimmunerkrankungen (AIBD) konzentrierten sich vor allem auf eine einzige Krankheitsentität oder nur eine Krankheitsgruppe; nur in wenigen Studien wurde die Inzidenz verschiedener AIBD untersucht. Bei der vorliegenden Studie war es unser Ziel, das gesamte Spektrum der AIBD zu betrachten, die Inzidenz der häufigsten AIBD zu ermitteln und die zeitlichen Trends ihres Auftretens in Zentralserbien über einen Zeitraum von 20 Jahren zu untersuchen.

    Methoden: Wir rekrutierten retrospektiv 1161 AIBD-Fälle, die in Zentralserbien von Januar 1991 bis Dezember 2010 neu diagnostiziert wurden. Read More

    Topically Applied Hsp90 Blocker 17AAG Inhibits Autoantibody-Mediated Blister-Inducing Cutaneous Inflammation.
    J Invest Dermatol 2017 Feb 19;137(2):341-349. Epub 2016 Sep 19.
    Department of Dermatology, University of Lübeck, Lübeck, Germany. Electronic address:
    Cell stress-inducible Hsp90 has been recognized as key player in mediating inflammatory responses. Although its systemic blockade was successfully used to treat autoimmune diseases in preclinical models, efficacy of a topical route of Hsp90 inhibitor administration has so far not been evaluated in chronic inflammatory and autoimmune-mediated dermatoses. Here, effects of the Hsp90 blocker 17-allylamino-demethoxygeldanamycin (17AAG) applied topically to the skin were determined in experimental inflammatory epidermolysis bullosa acquisita (EBA), an anti-type VII collagen autoantibody-induced blistering skin disease. Read More

    [Involvement of mucous membranes in autoimmune bullous diseases].
    Hautarzt 2016 Oct;67(10):774-779
    Klinik und Poliklinik für Dermatologie, Universitätsklinikum Dresden, Fetscherstr. 74, 01307, Dresden, Deutschland.
    Autoimmune bullous diseases are characterized by intraepidermal or subepidermal autoantibody deposition that leads to blisters and secondary erosion. Mucous membranes are frequently affected in pemphigus vulgaris and always involved in cicatricial and mucosal pemphigoid. Mucosal lesions are detected less frequently in patients with bullous pemphigoid or epidermolysis bullosa acquisita. Read More

    Clinical features and diagnosis of epidermolysis bullosa acquisita.
    Expert Rev Clin Immunol 2017 Feb 8;13(2):157-169. Epub 2016 Sep 8.
    a Department of Dermatology , University of Lübeck , Lübeck , Germany.
    Introduction: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disease of skin and mucous membranes. EBA is caused by autoantibodies against type VII collagen, which is a major component of anchoring fibrils, attaching epidermis to dermis. Binding of autoantibodies to type VII collagen leads to skin fragility and, finally, blister formation. Read More

    In vivo enzymatic modulation of IgG antibodies prevents immune complex-dependent skin injury.
    Exp Dermatol 2017 Aug 15;26(8):691-696. Epub 2016 Dec 15.
    Department of Biology, Institute of Genetics, University of Erlangen-Nuremberg, Erlangen, Germany.
    IgG antibodies are potent inducers of proinflammatory responses by cross-linking Fc receptors on innate immune effector cells resulting in tissue injury. The recently discovered enzymes endoglycosidase S (EndoS) and IgG-degrading enzyme (IdeS) of Streptococcus pyogenes are able to modulate the interaction between IgG antibodies and the Fc receptors, by hydrolysis of the glycan associated with the heavy chain of the IgG molecule (EndoS), or cleavage in the hinge region of the heavy IgG chain (IdeS). In this work, we investigated their ability to inhibit damage mediated by skin-bound antibodies in vivo in two different experimental models, the Arthus reaction, and epidermolysis bullosa acquisita, an autoimmune blistering skin disease associated with autoantibodies against type VII collagen. Read More

    Coexistence of acquired hemophilia A and epidermolysis bullosa acquisita: Two case reports and published work review.
    J Dermatol 2017 Jan 11;44(1):76-79. Epub 2016 Aug 11.
    Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
    Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal bullous autoimmune disease. The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. Read More

    Cytoskeletal Regulation of Inflammation and Its Impact on Skin Blistering Disease Epidermolysis Bullosa Acquisita.
    Int J Mol Sci 2016 Jul 13;17(7). Epub 2016 Jul 13.
    Future Industries Institute, Regenerative Medicine, University of South Australia, Mawson Lakes 5095, Adelaide, Australia.
    Actin remodelling proteins regulate cytoskeletal cell responses and are important in both innate and adaptive immunity. These responses play a major role in providing a fine balance in a cascade of biological events that results in either protective acute inflammation or chronic inflammation that leads to a host of diseases including autoimmune inflammation mediated epidermolysis bullosa acquisita (EBA). This review describes the role of the actin cytoskeleton and in particular the actin remodelling protein called Flightless I (Flii) in regulating cellular inflammatory responses and its subsequent effect on the autoimmune skin blistering disease EBA. Read More

    Childhood Epidermolysis Bullosa Acquisita: Confirmation of Diagnosis by Skin Deficient in Type VII Collagen, Enzyme-linked Immunosorbent Assay, and Immunoblotting.
    Indian J Dermatol 2016 May-Jun;61(3):329-32
    Department of Dermatology, University of Lübeck, Lübeck, Germany.
    Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disorder characterized by autoantibodies against Type VII collagen. It usually affects adults; childhood EBA is rare. We describe a 10-year-old girl presenting with recurrent tense blisters predominantly on legs, dorsa of hands and feet accompanied by oral erosions since the age of 5 years. Read More

    Clinical and immunological studies for 105 Japanese seropositive patients of epidermolysis bullosa acquisita examined at Kurume University.
    Expert Rev Clin Immunol 2016 Aug 16;12(8):895-902. Epub 2016 Jun 16.
    a Department of Dermatology , Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology , Fukuoka , Japan.
    Objectives: Using our serological diagnostic criteria, we selected 105 Japanese patients with epidermolysis bullosa acquisita (EBA), an autoimmune bullous disease (AIBD) reacting with type VII collagen, from our cohort of 5063 AIBD patients.

    Methods: We examined the patients clinically and immunologically.

    Results: We found diversity of clinical manifestations in both cutaneous and oral mucosal lesions and a high rate of inflammatory-type EBA patients in Japan. Read More

    Childhood epidermolysis bullosa acquisita during squaric acid dibutyl ester immunotherapy for alopecia areata.
    Br J Dermatol 2017 Feb 8;176(2):491-494. Epub 2016 Dec 8.
    Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS, via dei Monti di Creta 104, 00167, Rome, Italy.
    Epidermolysis bullosa acquisita (EBA) is a rare acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. Although EBA manifests more frequently in adults, it can occur in childhood. We describe a 6-year-old boy who developed the inflammatory variant of EBA shortly after initiation of immunotherapy with squaric acid dibutyl ester (SADBE) for scalp alopecia areata. Read More

    Laboratory Diagnosis and Clinical Profile of Anti-p200 Pemphigoid.
    JAMA Dermatol 2016 Aug;152(8):897-904
    Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
    Importance: Anti-p200 pemphigoid is a rare subepidermal autoimmune blistering disease characterized by autoantibodies against a 200-kDa protein in the basement membrane zone. Anti-p200 pemphigoid is probably often misdiagnosed because of low availability of diagnostic assays and expertise and classified as bullous pemphigoid or epidermolysis bullosa acquisita.

    Objective: To clinically characterize patients with anti-p200 pemphigoid, identified by using indirect immunofluorescence microscopy on skin substrates deficient in type VII collagen and laminin-332 (knockout analysis), to validate this technique by immunoblot with dermal extract, and to incorporate direct immunofluorescence serration pattern analysis in the diagnostic algorithm. Read More

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