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    Epidermolysis bullosa acquisita.
    Dermatol Online J 2017 Dec 15;23(12). Epub 2017 Dec 15.
    New York University, New York.
    Epidermolysis bullosa acquisita (EBA) is a rare, acquired subepidermal blistering disease. EBA is characterized by autoantibodies to collagen VII,which serves to link the epidermis to the dermis. The two most common presentations of EBA are classical noninflammatory EBA and bullous pemphigoid-like EBA. Read More

    Treatment of Autoimmune Bullous Disorders in Pregnancy.
    Am J Clin Dermatol 2018 Feb 2. Epub 2018 Feb 2.
    Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA.
    Autoimmune bullous diseases (AIBD), including pemphigus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, and pemphigoid gestationis, pose significant therapeutic challenges, especially in pregnant and post-partum breastfeeding patients or those planning to conceive. Data on the safety and efficacy of therapeutic interventions during the perinatal period are lacking because randomized controlled trials are typically not performed in this setting. However, many of the treatments for AIBD are also used in other diseases, so data can be extrapolated from studies or case reports in these other patient populations. Read More

    Epidermal aspects of type VII collagen: Implications for dystrophic epidermolysis bullosa and epidermolysis bullosa acquisita.
    J Dermatol 2018 Jan 20. Epub 2018 Jan 20.
    Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
    Type VII collagen (COL7), a major component of anchoring fibrils in the epidermal basement membrane zone, has been characterized as a defective protein in dystrophic epidermolysis bullosa and as an autoantigen in epidermolysis bullosa acquisita. Although COL7 is produced and secreted by both epidermal keratinocytes and dermal fibroblasts, the role of COL7 with regard to the epidermis is rarely discussed. This review focuses on COL7 physiology and pathology as it pertains to epidermal keratinocytes. Read More

    Response to: 'Human orf complicated by epidermolysis acquisita'.
    Br J Dermatol 2017 Dec 26. Epub 2017 Dec 26.
    The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York.
    Dear editor, we read with great interest the article 'Human orf complicated by epidermolysis bullosa acquisita' by Zeulgaray and colleagues.Recently, a Moroccan patient who contracted orf (fig. 1a) after slaughtering a sheep during Eid Al-Adha was admitted to our hospital and developed a similar pruritic vesiculobullous eruption predominantly on the hands and forearms (fig. Read More

    Acute renal failure in a patient with epidermolysis bullosa acquisita.
    An Bras Dermatol 2017 ;92(5 Suppl 1):14-16
    Department of Dermatology, Shandong Provincial Hospital for Skin Diseases, Shandong University, Shandong , China.
    Epidermolysis bullosa acquisita is a severe autoimmune subepidermal bullous disease. In this report, we described for the first time a patient with epidermolysis bullosa acquisita who developed acute renal failure. There is a possibility that epidermolysis bullosa acquisita and acute renal failure's pathogenesis shared some common autoimmune pathways. Read More

    Case of epidermolysis bullosa acquisita with concomitant anti-laminin-332 antibodies.
    J Dermatol 2017 Dec 4. Epub 2017 Dec 4.
    Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
    Subepidermal autoimmune blistering disease including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, anti-laminin-γ1 pemphigoid, linear immunoglobulin A bullous disease and epidermolysis bullosa acquisita (EBA), are all characterized by direct immunofluorescence microscopy or immunoglobulin deposition on the basement membrane zone. Among them, EBA is a rare acquired subepidermal autoimmune blistering disease of the skin and mucous membranes reactive with type VII collagen, a major component of the epidermal basement membrane zone. Anti-laminin-332-type mucous membrane pemphigoid has pathogenic autoantibodies against laminin-332, which is a basement membrane heterotrimeric protein composed of α3, β3 and γ2 laminin chains. Read More

    Epidermolysis Bullosa Acquisita in an Adult Patient with Previously Unrecognized Mild Dystrophic EB and Biallelic COL7A1 Mutations.
    Acta Derm Venereol 2017 Nov 28. Epub 2017 Nov 28.
    Circulating anti-type VII collagen autoantibodies are frequently detected in patients with recessive dystrophic epidermolysis bullosa (RDEB). However, evidence supporting their pathogenic role in inducing epidermolysis bullosa acquisita (EBA) has been provided for only 1 individual with dominant dystrophic epidermolysis bullosa (DDEB). We describe here a patient who presented with dystrophic toenails since early childhood and developed trauma-induced skin blisters and oral erosions at age 26 years. Read More

    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

    Subepidermal autoimmune bullous diseases: overview, epidemiology, and associations.
    Immunol Res 2018 Feb;66(1):6-17
    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 16. Epub 2017 Nov 16.
    Department of Dermatology, Center for Blistering Diseases, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. Electronic address:
    Background: Direct immunofluorescence (DIF) microscopy of a skin biopsy specimen is the reference standard for the diagnosis of pemphigoid diseases (PDs). Serration pattern analysis enables the differentiation of epidermolysis bullosa acquisita (EBA) from other PDs using DIF microscopy alone. However, practice gaps need to be addressed in order to implement this technique in the routine diagnostic procedure. 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.

    Calcitriol Treatment Ameliorates Inflammation and Blistering in Mouse Models of Epidermolysis Bullosa Acquisita.
    J Invest Dermatol 2018 Feb 20;138(2):301-309. Epub 2017 Sep 20.
    Department of Dermatology, University of Lübeck, Germany.
    A link between hypovitaminosis D and development of autoimmune bullous disorders has been suggested recently, but this association has not been elaborated experimentally. 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 Dec 7;26(12):1199-1206. Epub 2017 Nov 7.
    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 Dec;26(12):1207-1213
    Institute of Anatomy, University of Lübeck, 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

    CD11b-deficient mice exhibit an increased severity in the late phase of antibody transfer-induced experimental epidermolysis bullosa acquisita.
    Exp Dermatol 2017 Dec;26(12):1175-1178
    Xiamen-Borstel Joint Laboratory of Autoimmunity, Medical College of Xiamen University, Xiamen, China.
    CD11b, the α-chain of βintegrin Mac-1, is involved in many activation processes of phagocytes. Depending on the respective autoimmune disorder, CD11b has been shown to exert pro-inflammatory functions or be dispensable in their pathogenesis. Here, we investigated the role of CD11b in the pathogenesis of experimental epidermolysis bullosa acquisita (EBA), an autoimmune skin blistering disease mediated by autoantibodies to type VII collagen. 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 Dec 2;26(12):1163-1170. Epub 2017 Nov 2.
    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 2018 Feb 3;178(2):350-356. Epub 2018 Jan 3.
    Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, U.S.A.
    Colchicine is a 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 U. Read More

    Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.
    Clin Rev Allergy Immunol 2018 Feb;54(1):26-51
    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 2018 Jan 14;178(1):207-214. Epub 2017 Dec 14.
    Department of Dermatology, University of Heidelberg, Heidelberg, Germany.
    Background: Neutrophil (polymorphonuclear) granulocytes (PMN) have been shown to contribute to the pathogenesis of psoriasis by releasing interleukin-17 and LL37-DNA complexes via neutrophil extracellular traps (NETs), 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 2018 Jan/Feb;22(1):22-24. Epub 2017 Jul 18.
    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 multicentre analysis.
    Br J Dermatol 2017 Dec 1;177(6):1683-1692. Epub 2017 Dec 1.
    Department of Dermatology and Allergology, Philipps-University, Marburg, D-35043, Germany.
    Background: Epidermolysis bullosa acquisita (EBA) 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 scarring 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 2018 Feb 17;178(2):547-550. Epub 2017 Oct 17.
    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 Dec 9;26(12):1179-1186. Epub 2017 May 9.
    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 Band 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 B(LTB) 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

    Elucidating the interplay between IgG-Fc valency and FcγR activation for the design of immune complex inhibitors.
    Sci Transl Med 2016 Nov;8(365):365ra158
    Momenta Pharmaceuticals, 675 West Kendall Street, Cambridge, MA 02142, USA.
    Autoantibody immune complex (IC) activation of Fcγ receptors (FcγRs) is a common pathogenic hallmark of multiple autoimmune diseases. Given that the IC structural features that elicit FcγR activation are poorly understood and the FcγR system is highly complex, few therapeutics can directly block these processes without inadvertently activating the FcγR system. To address these issues, the structure activity relationships of an engineered panel of multivalent Fc constructs were evaluated using sensitive FcγR binding and signaling cellular assays. 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

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