942 results match your criteria Epidermolysis Bullosa Acquisita


Gastrointestinal involvement of primary skin diseases.

J Eur Acad Dermatol Venereol 2020 May 26. Epub 2020 May 26.

Center for Research & Development, Kaohsiung Municipal Siaogang Hospital, Kaohsiung, Taiwan.

Less is known about gastrointestinal (GI) involvement of primary skin diseases due to the difference in embryology, histology, microbiology and physiology between integument and alimentary tract. Oesophagus, following the oropharyngeal mucosa, is the most common GI segment affected by primary skin diseases, especially by eosinophilic oesophagitis, lichen planus and autoimmune bullous dermatoses like pemphigus vulgaris, mucosal membrane pemphigoid and epidermolysis bullosa acquisita. Eosinophilic oesophagitis is an emerging chronic atopic disease with oesophageal dysfunction as the typical presentation, and oesophageal narrowing, rings and stricture as late complications. Read More

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http://dx.doi.org/10.1111/jdv.16676DOI Listing

Propranolol is an effective topical and systemic treatment option for experimental epidermolysis bullosa acquisita.

J Invest Dermatol 2020 May 22. Epub 2020 May 22.

Lübeck Institute of Experimental Dermatology, University of Lübeck, Germany. Electronic address:

Propranolol is a ß-adrenoreceptor type 2 (ADRB2) blocker that regulates heart muscle contractions, smooth muscle relaxation and glycogenolysis. In addition, an increasing number of applications in dermatology have been described; most prominently, the use as a first-line treatment for infantile hemangiomas. We here show that propranolol enhances IL-8-induced neutrophil chemotaxis and reduces the release of reactive oxygen species (ROS) after immune complex (IC) stimulation. Read More

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http://dx.doi.org/10.1016/j.jid.2020.04.025DOI Listing

Epidermolysis Bullosa Acquisita: A Case Report.

Am J Case Rep 2020 Apr 20;21:e919432. Epub 2020 Apr 20.

Division of Dermatology, U.S. Naval Hospital Guam, Agana Heights, Guam.

BACKGROUND Epidermolysis bullosa acquisita is a rare, subepithelial bullous disorder, which is distinguished from other autoimmune blistering diseases by the production of antibodies against type VII collagen. CASE REPORT Here, we describe the case of a 79-year-old male resident of the Northern Mariana Islands who presented to the clinic with multiple blistering skin lesions. CONCLUSIONS The primary focus of treatment is to prevent disease progression and serious complications of scarring (including blindness and respiratory obstruction) by avoiding physical trauma and suppressing the immune systems with agents, including corticosteroids, colchicine, dapsone, methotrexate, and cyclophosphamide. Read More

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http://dx.doi.org/10.12659/AJCR.919432DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7193220PMC

[Epidermolysis bullosa acquisita with Brunsting-Perry type pemphigoid: Diagnostic and therapeutic difficulties].

Ann Dermatol Venereol 2020 Jun - Jul;147(6-7):439-445. Epub 2020 Mar 31.

Service de dermatologie et allergologie, CHRU Nancy, 51, boulevard Albert-Premier, 54000 Nancy, France.

Background: Epidermolysis bullosa acquisita (EBA) is a rare auto-immune blistering disease. We report a case of Brunsting-Perry pemphigoid diagnosed by immunoelectron microscopy (IEM).

Patients And Methods: A 46-year-old man presented very pruriginous vesicles on the face and neck present for 6 years and which were difficult to diagnose and treat. Read More

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http://dx.doi.org/10.1016/j.annder.2020.01.005DOI Listing

A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita.

Am J Clin Dermatol 2020 Mar 16. Epub 2020 Mar 16.

Department of Dermatology, University of Illinois at Chicago, 808 S. Wood St, RM377, Chicago, IL, 60612, USA.

Autoimmune blistering disease management can be challenging as treatment modalities vary greatly and no single standard of care exists. We consolidated the recommendations of international management guidelines in order to provide optimal management suggestions to physicians. A comprehensive literature search in PubMed/MEDLINE for published blistering disease management guidelines and consensus statements was conducted in November 2019. Read More

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http://dx.doi.org/10.1007/s40257-020-00513-3DOI Listing

Visualization of autoantibodies and neutrophils in vivo identifies novel checkpoints in autoantibody-induced tissue injury.

Sci Rep 2020 Mar 11;10(1):4509. Epub 2020 Mar 11.

Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

In several autoimmune diseases, e.g., pemphigoid disease (PD), autoantibodies are the direct cause of pathology. Read More

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http://dx.doi.org/10.1038/s41598-020-60233-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7066238PMC

Serration pattern analysis as a tool for the diagnosis of immunoglobulin A-mediated epidermolysis bullosa acquisita.

J Dermatol 2020 May 3;47(5):e198-e199. Epub 2020 Mar 3.

Section of Dermatology, Department of Health Sciences, University of Florence, Florence, Italy.

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http://dx.doi.org/10.1111/1346-8138.15161DOI Listing

Dyshidrosiform Bullous Pemphigoid: Case Reports and Review.

Authors:
Philip R Cohen

Cureus 2020 Jan 11;12(1):e6630. Epub 2020 Jan 11.

Dermatology, San Diego Family Dermatology, San Diego, USA.

Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla, and groin. It is caused by circulating and tissue-bound autoantibodies directed against bullous pemphigoid antigen 1 or bullous pemphigoid antigen 2 or both. Dyshidrosiform bullous pemphigoid is a rare variant of bullous pemphigoid, and it usually presents as itchy, potentially hemorrhagic, or purpuric blisters on the palms and/or soles of elderly individuals; subsequently, typical bullous lesions of bullous pemphigoid appear on other body sites. Read More

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http://dx.doi.org/10.7759/cureus.6630DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7008730PMC
January 2020

Epidermal Damage Induces Th1 Polarization and Defines the Site of Inflammation in Murine Epidermolysis Bullosa Acquisita.

J Invest Dermatol 2020 Feb 11. Epub 2020 Feb 11.

Institute of Anatomy, University of Lübeck, Lübeck, Germany. Electronic address:

Epidermolysis bullosa acquisita is an autoimmune skin disease characterized by subepidermal blisters. The pathogenesis is mediated by deposits of autoantibodies directed against type VII collagen in the skin, but the sequence of events regulating the localization of skin blisters is not fully understood. In this study, using the immunization-induced mouse model of epidermolysis bullosa acquisita, we demonstrate that epidermal disruption induces not only an infiltration of CD4 T cells but also a T helper type 1 phenotype as it has been described for delayed-type hypersensitivity reactions. Read More

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http://dx.doi.org/10.1016/j.jid.2020.01.022DOI Listing
February 2020

Drug Development in Pemphigoid Diseases.

Acta Derm Venereol 2020 02 12;100(5):adv00055. Epub 2020 Feb 12.

Lübeck Institute of Experimental Dermatology, University of Lübeck, 23562 Lübeck, Germany.

Pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes. They are caused by autoantibodies targeting adhesion molecules located at the dermal-epidermal junction. While the diagnostics of pemphigoid diseases and insights into their pathogenesis have improved significantly, the development of novel treatments that are effective and safe remains an unmet medical need. Read More

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http://dx.doi.org/10.2340/00015555-3400DOI Listing
February 2020

Structural and biophysical characterization of the type VII collagen vWFA2 subdomain leads to identification of two binding sites.

FEBS Open Bio 2020 Apr 14;10(4):580-592. Epub 2020 Mar 14.

Institute of Chemistry and Metabolomics, University of Lübeck, Germany.

Type VII collagen is an extracellular matrix protein, which is important for skin stability; however, detailed information at the molecular level is scarce. The second vWFA (von Willebrand factor type A) domain of type VII collagen mediates important interactions, and immunization of mice induces skin blistering in certain strains. To understand vWFA2 function and the pathophysiological mechanisms leading to skin blistering, we structurally characterized this domain by X-ray crystallography and NMR spectroscopy. Read More

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http://dx.doi.org/10.1002/2211-5463.12807DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7137805PMC

Fcγ Receptor IIB Controls Skin Inflammation in an Active Model of Epidermolysis Bullosa Acquisita.

Front Immunol 2019 14;10:3012. Epub 2020 Jan 14.

Institute for Systemic Inflammation Research, University of Lübeck, Lübeck, Germany.

Epidermolysis bullosa acquisita (EBA) is an autoimmune skin blistering disease characterized by IgG autoantibodies (aAb) against type VII collagen (COL7). The mechanisms controlling the formation of such aAbs and their effector functions in the skin tissue are incompletely understood. Here, we assessed whether the inhibitory IgG Fc receptor, FcγRIIB, controls the development of autoimmune skin blistering disease in an active model of EBA. Read More

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http://dx.doi.org/10.3389/fimmu.2019.03012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6971089PMC
January 2020

Treatment with anti-neonatal Fc receptor (FcRn) antibody ameliorates experimental epidermolysis bullosa acquisita in mice.

Br J Pharmacol 2020 May 6;177(10):2381-2392. Epub 2020 Mar 6.

Lübeck Institute of Experimental Dermatology, and Center for Research on Inflammation of the Skin, University of Lübeck, Lübeck, Germany.

Background And Purpose: Pemphigus and pemphigoid diseases are characterized and caused predominantly by IgG autoantibodies targeting structural proteins of the skin. Their current treatment relies on general and prolonged immunosuppression that causes severe adverse events, including death. Hence, novel safe and more effective treatments are urgently needed. Read More

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http://dx.doi.org/10.1111/bph.14986DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7174883PMC

Epidermolysis Bullosa Acquisita: A Case Report of a Rare Clinical Phenotype and a Review of Literature.

Cureus 2019 Dec 15;11(12):e6386. Epub 2019 Dec 15.

Oncologic Dermatology, Elias Emergency University Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, ROU.

Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal bullous disorder of the skin and mucous membranes. The disease results from the production of immunoglobulin G (IgG) antibodies against type-VII collagen, a major component of anchoring filaments in the dermal-epithelial junction. The disease has two major forms of presentation: the classical (non-inflammatory) type and the inflammatory type. Read More

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http://dx.doi.org/10.7759/cureus.6386DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6957238PMC
December 2019

Efficacy of intravenous immunoglobulins for laryngopharyngeal lesions and upper airway obstruction in epidermolysis bullosa acquisita.

J Eur Acad Dermatol Venereol 2020 Mar 3;34(3):e131-e133. Epub 2019 Dec 3.

Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

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http://dx.doi.org/10.1111/jdv.16080DOI Listing

Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita).

J Dermatol 2019 Dec 24;46(12):1102-1135. Epub 2019 Oct 24.

Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

The pemphigoid group is a category of autoimmune subepidermal blistering diseases in which autoantibodies deposit linearly at the epidermal basement membrane zone (BMZ). The main subtypes of pemphigoid mediated by immunoglobulin G autoantibodies are bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA). To establish the first guidelines approved by the Japanese Dermatological Association for the management of pemphigoid diseases, the Committee for Guidelines for the Management of Pemphigoid Diseases (Including EBA) was founded as part of the Study Group for Rare Intractable Skin Diseases under the Ministry of Health, Labor and Welfare Research Project on Overcoming Intractable Diseases. Read More

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http://dx.doi.org/10.1111/1346-8138.15111DOI Listing
December 2019

Brunsting-Perry Pemphigoid as Differential Diagnosis of Nonmelanoma Skin Cancer.

Authors:
Gerhard Eichhoff

Cureus 2019 Aug 16;11(8):e5400. Epub 2019 Aug 16.

Dermatology Service, Wellington Regional Hospital, Wellington, NZL.

Brunsting-Perry pemphigoid is a rare autoimmune blistering skin disease. Similar to nonmelanoma skin cancers, Brunsting-Perry pemphigoid has a predilection for the head and neck. Herein, a case of solitary Brunsting-Perry pemphigoid treated as cutaneous squamous cell carcinoma (SCC) with subsequent excision is reported. Read More

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http://dx.doi.org/10.7759/cureus.5400DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6793596PMC
August 2019
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Successful treatment of refractory epidermolysis bullosa acquisita with intravenous immunoglobulin and dapsone.

Cutis 2019 Aug;104(2):E20-E21

Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock, USA.

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August 2019
1 Read

Alopecia in Autoimmune Blistering Diseases: A Systematic Review of Pathogenesis and Clinical Features of Disease.

Skin Appendage Disord 2019 Aug 10;5(5):263-275. Epub 2019 Jul 10.

Department of Dermatology, St. George Hospital, Sydney, New South Wales, Australia.

Background: Autoimmune blistering diseases (AIBD) are characterised by the body's production of autoantibodies against structural proteins in the epidermis and/or the basement membrane on cutaneous and mucosal surfaces. Alopecia is a complication of AIBD that has generally been overlooked in patients with severe blistering diseases because it is regarded as a cosmetic issue. Yet recent research into quality of life tools has found that stigmatisation by appearance plays a significant role in blistering diseases. Read More

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http://dx.doi.org/10.1159/000496836DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6751435PMC
August 2019
7 Reads

Bullous Diseases in Children: A Review of Clinical Features and Treatment Options.

Paediatr Drugs 2019 Oct;21(5):345-356

Department of Dermatology, University of Minnesota, 240 Phillips-Wangensteen Building, 516 Delaware Street Southeast, Minneapolis, MN, 55455, USA.

Bullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Read More

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http://dx.doi.org/10.1007/s40272-019-00349-3DOI Listing
October 2019
5 Reads

Dual inhibition of complement factor 5 and leukotriene B4 synergistically suppresses murine pemphigoid disease.

JCI Insight 2019 08 8;4(15). Epub 2019 Aug 8.

Department of Dermatology, Allergy, and Venereology.

The treatment of most autoimmune diseases still relies on systemic immunosuppression and is associated with severe side effects. The development of drugs that more specifically abrogate pathogenic pathways is therefore most desirable. In nature, such specificity is exemplified, e. Read More

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http://dx.doi.org/10.1172/jci.insight.128239DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6693894PMC
August 2019
2 Reads

From bench to bedside: evolving therapeutic targets in autoimmune blistering disease.

J Eur Acad Dermatol Venereol 2019 Dec 14;33(12):2239-2252. Epub 2019 Oct 14.

Department of Dermatology, University of Illinois at Chicago, Chicago, IL, USA.

Autoimmune blistering diseases comprise a group of heterogenous conditions characterized by the loss of tolerance and subsequent development of autoantibodies targeting epidermal and subepidermal adhesion proteins. Blisters and erosions form on the skin and mucous membranes leading to significant morbidity and mortality. Traditional therapies rely on systemic immunosuppression. Read More

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http://dx.doi.org/10.1111/jdv.15816DOI Listing
December 2019
3 Reads

Proteases in Pemphigoid Diseases.

Front Immunol 2019 26;10:1454. Epub 2019 Jun 26.

International Collaboration On Repair Discoveries (ICORD), Vancouver Coastal Health Research Institute (VCHRI), Vancouver, BC, Canada.

Pemphigoid diseases are a subgroup of autoimmune skin diseases characterized by widespread tense blisters. Standard of care typically involves immunosuppressive treatments, which may be insufficient and are often associated with significant adverse events. As such, a deeper understanding of the pathomechanism(s) of pemphigoid diseases is necessary in order to identify improved therapeutic approaches. Read More

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http://dx.doi.org/10.3389/fimmu.2019.01454DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6607946PMC
June 2019
2 Reads

Complement Activation in Autoimmune Bullous Dermatoses: A Comprehensive Review.

Front Immunol 2019 26;10:1477. Epub 2019 Jun 26.

Department of Pathology, Erasmus Medical Center Rotterdam, Rotterdam, Netherlands.

Autoimmune bullous dermatoses (AIBD) are characterized by circulating autoantibodies that are either directed against epidermal antigens or deposited as immune complexes in the basement membrane zone (BMZ). The complement system (CS) can be activated by autoantibodies, thereby triggering activation of specific complement pathways. Local complement activation induces a pathogenic inflammatory response that eventually results in the formation of a sub- or intraepidermal blister. Read More

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http://dx.doi.org/10.3389/fimmu.2019.01477DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6606728PMC
June 2019
3 Reads

High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease.

Front Immunol 2019 11;10:1090. Epub 2019 Jun 11.

Department of Dermatology, University of Heidelberg, Heidelberg, Germany.

The immunomodulatory potential and low incidence of severe side effects of high-dose intravenous immunoglobulin (IVIg) treatment led to its successful application in a variety of dermatological autoimmune diseases over the last two decades. IVIg is usually administered at a dose of 2 g per kg body weight distributed over 2-5 days every 4 weeks. They are most commonly used as a second- or third-line treatment in dermatological autoimmune disease (pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, dermatomyositis, systemic vasculitis, and systemic lupus erythematosus). Read More

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http://dx.doi.org/10.3389/fimmu.2019.01090DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6579842PMC
June 2019
10 Reads

Epidermolysis bullosa acquisita: A comprehensive review.

Autoimmun Rev 2019 Aug 7;18(8):786-795. Epub 2019 Jun 7.

Department of Dermatology, University of Illinois at Chicago, Chicago, IL, United States of America. Electronic address:

Epidermolysis bullosa acquisita is a rare autoimmune blistering disease which results in vesicle and bullae formation on the skin and erosions on the mucous membranes. EBA is mediated by autoantibodies to collagen VII. Clinically, it can present with numerous phenotypes, though the most common are the mechanobullous and inflammatory variants. Read More

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http://dx.doi.org/10.1016/j.autrev.2019.06.007DOI Listing
August 2019
15 Reads

Optimization of reference gene panels for gene expression analysis in preclinical models of inflammatory skin diseases.

Exp Dermatol 2019 08 3;28(8):985-988. Epub 2019 Jul 3.

Department of Dermatology, Allergy, and Venereology, University of Lübeck, Lübeck, Germany.

Reverse transcriptase qPCR is the most common method to determine and compare mRNA expression levels and relies on normalization using reference genes. The expression levels of the latter, however, are themselves often variable between experimental conditions, thus compromising the results. Using the geNorm algorithm, we have examined seven genes with respect to their suitability as reference genes for gene analysis in mouse models of skin inflammation, using the antibody transfer model of epidermolysis bullosa acquisita and in the Aldara -induced psoriasiform dermatitis. Read More

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http://dx.doi.org/10.1111/exd.13989DOI Listing
August 2019
4 Reads

The Sphingosine-1-Phosphate Receptor Modulator Fingolimod Aggravates Murine Epidermolysis Bullosa Acquisita.

J Invest Dermatol 2019 11 7;139(11):2381-2384.e3. Epub 2019 Jun 7.

Department of Dermatology, Allergy, and Venereology, University of Lübeck, Lübeck, Germany; Center for Research on Inflammation of the Skin (CRIS), University of Lübeck, Lübeck, Germany. Electronic address:

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http://dx.doi.org/10.1016/j.jid.2019.03.1159DOI Listing
November 2019
10 Reads

Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology.

An Bras Dermatol 2019 Apr 30;94(2 Suppl 1):33-47. Epub 2019 Jun 30.

Department of Dermatology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. For each of these dermatoses, a literature review covering all therapeutic options was performed. Read More

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http://www.scielo.br/scielo.php?script=sci_arttext&pid=S
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http://dx.doi.org/10.1590/abd1806-4841.2019940207DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6544032PMC
April 2019
9 Reads

Skin Barrier and Autoimmunity-Mechanisms and Novel Therapeutic Approaches for Autoimmune Blistering Diseases of the Skin.

Front Immunol 2019 14;10:1089. Epub 2019 May 14.

Regenerative Medicine Laboratory, Future Industries Institute, University of South Australia, Adelaide, SA, Australia.

One of the most important functions of the skin besides regulating internal body temperature includes formation of the barrier between the organism and the external environment, hence protecting against pathogen invasion, chemical and physical assaults and unregulated loss of water and solutes. Disruption of the protective barrier is observed clinically in blisters and erosions of the skin that form in autoimmune blistering diseases where the body produces autoantibodies against structural proteins of the epidermis or the epidermal-dermal junction. Although there is no cure for autoimmune skin blistering diseases, immune suppressive therapies currently available offer opportunities for disease management. Read More

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http://dx.doi.org/10.3389/fimmu.2019.01089DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6530337PMC
June 2020
6 Reads

Ocular Manifestations and Management of Autoimmune Bullous Diseases.

J Ophthalmic Vis Res 2019 Apr-Jun;14(2):195-210

Ocular Tissue Engineering Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Autoimmune bullous diseases with ocular involvement consist of a group of systemic entities that are characterized by formation of autoantibodies against the proteins of the epithelial basement membrane zone of the conjunctiva. Mostly, the elderly are affected by these diseases. The characteristic patterns of mucocutaneous involvement and the specific tissue components targeted by these autoantibodies are differentiating features of these diseases. Read More

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http://dx.doi.org/10.4103/jovr.jovr_86_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6504727PMC
May 2019
8 Reads

Mucous membrane pemphigoid and oral blistering diseases.

Clin Exp Dermatol 2019 Oct 18;44(7):732-739. Epub 2019 May 18.

Oral Medicine, Dental Institute, Guy's and St Thomas' NHS Foundation Trust, London, UK.

The autoimmune blistering disorders present with variable frequency in the oral cavity. Recognition of their key clinical features at presentation is important, as there are many causes of oral ulceration. Careful history-taking, clinical examination, an understanding of pathogenesis and appropriate investigations are essential. Read More

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http://dx.doi.org/10.1111/ced.13996DOI Listing
October 2019
13 Reads

Pemphigoid variants affecting the skin.

Clin Exp Dermatol 2019 Oct 16;44(7):721-727. Epub 2019 May 16.

Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

Pemphigoid diseases are autoimmune subepidermal blistering diseases affecting the skin and mucous membranes, which are caused by autoantibodies targeting structural hemidesmosomal proteins or hemidesmosome-associated proteins. Variants of pemphigoid can be differentiated based on targeted antigens and clinical aspects. In this review, we will discuss pemphigoid variants that predominantly affect the skin, and provide clinicians with clues to diagnosis. Read More

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http://dx.doi.org/10.1111/ced.13984DOI Listing
October 2019
2 Reads

Ocular involvement in epidermolysis bullosa acquisita with long-term follow-up.

Br J Ophthalmol 2020 Feb 14;104(2):235-240. Epub 2019 May 14.

Cornea, External Disorders and Refractive Surgery, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France

Background/aims: To describe the ocular manifestations associated with epidermolysis bullosa acquisita (EBA).

Methods: This retrospective study was conducted at a tertiary bullous disease clinic. Consecutive patients were enrolled with biopsy proven diagnosis of EBA, with ocular involvement and a follow-up of at least 36 months. Read More

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http://dx.doi.org/10.1136/bjophthalmol-2019-313960DOI Listing
February 2020
11 Reads

Review of autoimmune blistering diseases: the Pemphigoid diseases.

J Eur Acad Dermatol Venereol 2019 Sep 11;33(9):1685-1694. Epub 2019 Jul 11.

St George Hospital, Sydney, Australia.

Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub-epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/jdv.15679
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http://dx.doi.org/10.1111/jdv.15679DOI Listing
September 2019
45 Reads

Type VII collagen IgE autoantibodies in epidermolysis bullosa acquisita: more common than suspected.

Authors:
R J Ludwig

Br J Dermatol 2019 05;180(5):981-983

Lübeck Institute of Experimental Dermatology and Center for Research on Inflammation of the Skin, University of Lübeck, Lübeck, Germany.

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http://dx.doi.org/10.1111/bjd.17770DOI Listing
May 2019
4 Reads

Possible involvement of IgE antibody in epidermolysis bullosa acquisita: detection and correlation.

Eur J Dermatol 2019 04;29(2):210-212

Department of Dermatology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8575, Japan.

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http://dx.doi.org/10.1684/ejd.2019.3497DOI Listing
April 2019
5 Reads

Milia within resolving bullous pemphigoid lesions.

Proc (Bayl Univ Med Cent) 2019 Jan 14;32(1):90-92. Epub 2019 Jan 14.

Texas A&M College of Medicine, College StationTexas.

Bullous pemphigoid (BP) is a blistering dermatosis characterized by an autoimmune response to two hemidesmosomal proteins, BP180 and BP230. We describe a case of an 80-year-old man diagnosed with BP by clinical features, histopathology, and immunosorbent assay who developed milia within resolving BP lesions. Milia formation during recovery is common in cases of mucous membrane pemphigoid and epidermolysis bullosa acquisita but has rarely been reported in cases of BP. Read More

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http://dx.doi.org/10.1080/08998280.2018.1528962DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6442898PMC
January 2019
28 Reads

Correction: Pathology in Practice: presumptive epidermolysis bullosa acquisita in a dog.

Authors:

J Am Vet Med Assoc 2019 Apr;254(8):918

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http://dx.doi.org/10.2460/javma.254.8.918DOI Listing
April 2019
2 Reads

[Epidermolysis bullosa acquisita].

Hautarzt 2019 Apr;70(4):265-270

Universitäts-Hautklinik Kiel, Klinik für Dermatologie, Venerologie und Allergologie, UKSH Kiel, Kiel, Deutschland.

Epidermolysis bullosa acquisita (EBA) is a rare acquired subepidermal bullous autoimmune dermatosis, associated with autoantibodies against collagen type VII, the most important component of dermal anchoring fibrils. Blister induction occurs after binding of autoantibodies to collagen type VII, leading to complement activation, recruitment of neutrophils and secretion of proteases. Clinically, the disease is mostly characterized by tense blisters on trauma-exposed body areas which heal with scarring (mechanobullous form of EBA). Read More

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http://dx.doi.org/10.1007/s00105-019-4387-7DOI Listing
April 2019
5 Reads

Treatment Update of Autoimmune Blistering Diseases.

Dermatol Clin 2019 Apr 14;37(2):215-228. Epub 2019 Feb 14.

Department of Medicine, University of Central Florida, Health Sciences Campus at Lake Nona, 2627 Northampton Avenue, Orlando, FL 32827-7408, USA. Electronic address:

The treatment of refractory autoimmune blistering diseases (AIBDs) has always been a challenge. Because randomized controlled trials are lacking, treatment has been based on analysis of anecdotal data. The last 2 decades has seen the use of rituximab become a conventional treatment in the therapeutic armamentarium of AIBDs, leading to its Food and Drug Administration indication for pemphigus vulgaris in 2018. Read More

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http://dx.doi.org/10.1016/j.det.2018.12.003DOI Listing
April 2019
10 Reads

Ocular Mucous Membrane Pemphigoid: Current State of Pathophysiology, Diagnostics and Treatment.

Ophthalmol Ther 2019 Mar 29;8(1):5-17. Epub 2019 Jan 29.

Department of Ophthalmology, Basel University Hospital, Basel, Switzerland.

Mucous membrane pemphigoid (MMP) is a systemic cicatrizing autoimmune disease that primarily affects orificial mucous membranes, such as the conjunctiva, the nasal cavity, the oropharynx, and the genitalia. Ocular involvement occurs in about 70% of all MMP cases. Ocular MMP (OcMMP) also encompasses the conditions linear immunoglobulin A disease, mucosal dominated epidermolysis bullosa acquisita, and anti-laminin 332/anti-epiligrin/anti-laminin 5 pemphigoid. Read More

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http://dx.doi.org/10.1007/s40123-019-0164-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393250PMC
March 2019
24 Reads

Steroid-induced Diabetes Complicating Treatment of Epidermolysis Bullosa Acquisita: A Preventable Treatment Complication Stresses the Importance of Primary Care Follow-up.

Cureus 2018 Nov 19;10(11):e3608. Epub 2018 Nov 19.

Internal Medicine, Louis Stokes Cleveland VA Medical Center/Case Western Reserve University School of Medicine, Cleveland, USA.

Epidermolysis bullosa acquisita is a rare autoimmune bullous disease involving the skin and mucosa, most commonly treated with systemic corticosteroids. This case illustrates the importance of counseling patients on medication side effects and ensuring close physician follow-up during an extended course of steroids. A 46-year-old man presented to the emergency department with weakness, fatigue, dizziness and polyuria in the setting of eight weeks of prednisone therapy for a flare-up of his bullous disease. Read More

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http://dx.doi.org/10.7759/cureus.3608DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343862PMC
November 2018
8 Reads

Epidermolysis Bullosa Acquisita: The 2019 Update.

Front Med (Lausanne) 2018 10;5:362. Epub 2019 Jan 10.

Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Patients with EBA suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes. Current treatment options rely on non-specific immunosuppression, which in many cases, does not lead to a remission of treatment. Read More

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http://dx.doi.org/10.3389/fmed.2018.00362DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335340PMC
January 2019
37 Reads

Elevation of serum interleukin-21 in patients with epidermolysis bullosa acquisita.

J Dermatol 2019 Mar 23;46(3):279-280. Epub 2019 Jan 23.

Department of Dermatology, Gangnam Severance Hospital, Seoul, Korea.

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http://doi.wiley.com/10.1111/1346-8138.14789
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http://dx.doi.org/10.1111/1346-8138.14789DOI Listing
March 2019
25 Reads

A Case Report of the Use of Rituximab and the Epidermolysis Bullosa Disease Activity Scoring Index (EBDASI) in a Patient with Epidermolysis Bullosa Acquisita with Extensive Esophageal Involvement.

Acta Dermatovenerol Croat 2018 Dec;26(4):325-328

Professor Dedee F. Murrell, MA, BM, FAAD, MD, FACD FRCP (Edin), Department of Dermatology St. George Hospital University of NSW Gray St, Kogarah, Sydney, Australia;

A 49-year-old man with recalcitrant mechanobullous epidermolysis bullosa acquisita (EBA) with significant esophageal involvement was treated with rituximab. EBA is a chronic autoimmune subepidermal bullous disease. It is characterized by skin fragility and scarring caused by circulating and tissue bound antibodies to type VII collagen. Read More

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December 2018
25 Reads

Detection of u-serrated patterns in direct immunofluorescence images of autoimmune bullous diseases by inhibition-augmented COSFIRE filters.

Int J Med Inform 2019 02 28;122:27-36. Epub 2018 Nov 28.

Bernoulli Institute for Mathematics, Computer Science, University of Groningen, The Netherlands.

Direct immunofluorescence (DIF) microscopy of a skin biopsy is used by physicians and pathologists to diagnose autoimmune bullous dermatoses (AIBD). This technique is the reference standard for diagnosis of AIBD, which is used worldwide in medical laboratories. For diagnosis of subepidermal AIBD (sAIBD), two different types of serrated pattern of immunodepositions can be recognized from DIF images, namely n- and u-serrated patterns. Read More

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http://dx.doi.org/10.1016/j.ijmedinf.2018.11.007DOI Listing
February 2019
9 Reads

Myeloid-Specific Deletion of Mcl-1 Yields Severely Neutropenic Mice That Survive and Breed in Homozygous Form.

J Immunol 2018 12 21;201(12):3793-3803. Epub 2018 Nov 21.

Department of Physiology, Semmelweis University School of Medicine, 1094 Budapest, Hungary;

Mouse strains with specific deficiency of given hematopoietic lineages provide invaluable tools for understanding blood cell function in health and disease. Whereas neutrophils are dominant leukocytes in humans and mice, there are no widely useful genetic models of neutrophil deficiency in mice. In this study, we show that myeloid-specific deletion of the Mcl-1 antiapoptotic protein in () mice leads to dramatic reduction of circulating and tissue neutrophil counts without affecting circulating lymphocyte, monocyte, or eosinophil numbers. Read More

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http://dx.doi.org/10.4049/jimmunol.1701803DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287103PMC
December 2018
8 Reads

Diagnosis of Autoimmune Blistering Diseases.

Front Med (Lausanne) 2018 2;5:296. Epub 2018 Nov 2.

Department of Dermatology, University of Lübeck, Lübeck, Germany.

Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Read More

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http://dx.doi.org/10.3389/fmed.2018.00296DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6224342PMC
November 2018
39 Reads