1,014 results match your criteria Epidermolysis Bullosa Acquisita


Current and Innovated Managements for Autoimmune Bullous Skin Disorders: An Overview.

J Clin Med 2022 Jun 19;11(12). Epub 2022 Jun 19.

Department of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807378, Taiwan.

Autoimmune bullous skin disorders are a group of disorders characterized by the formation of numerous blisters and erosions on the skin and/or the mucosal membrane, arising from autoantibodies against the intercellular adhesion molecules and the structural proteins. They can be classified into intraepithelial or subepithelial autoimmune bullous dermatoses based on the location of the targeted antigens. These dermatoses are extremely debilitating and fatal in certain cases, depending on the degree of cutaneous and mucosal involvement. Read More

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Epidermolysis bullosa acquisita.

An Bras Dermatol 2022 Jun 11. Epub 2022 Jun 11.

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

Epidermolysis bullosa acquisita is a rare autoimmune disease, characterized by the synthesis of anti-collagen VII autoantibodies, the main component of hemidesmosome anchoring fibrils. The antigen-antibody binding elicits a complex inflammatory response, which culminates in the loss of dermo-epidermal adhesion of the skin and/or mucous membranes. Skin fragility with bullae, erosions, and milia in areas of trauma characterizes the mechanobullous form of the disease. Read More

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Assessing quality of life in patients with autoimmune bullous diseases using the Persian version of Treatment of Autoimmune Bullous Disease Quality of Life questionnaire finds similar effects in women as men.

Int J Womens Dermatol 2022 Mar 21;8(1):e004. Epub 2022 Mar 21.

Autoimmune Bullous Disease Research Center, Razi Hospital, Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran.

In autoimmune bullous diseases (AIBDs), autoantibodies loosen molecular adhesions in the skin and/or mucosa and lead to blisters and erosions. Immunosuppressive drugs reduce mortality of the AIBD; therefore, patients will have to live longer with comorbidities.

Objective: This study aims to determine the quality of life of AIBD patients undergoing systemic treatment while investigating the survey's relationship with various factors. Read More

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The association of six autoimmune bullous diseases with thyroid disorders: a population-based study.

J Eur Acad Dermatol Venereol 2022 May 25. Epub 2022 May 25.

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

Background: The association of autoimmune bullous diseases (AIBDs) with thyroid disorders remains to be profoundly investigated.

Objective: To evaluate the epidemiological association between six AIBDs and thyroid disorders.

Methods: A population-based cross-sectional study enrolled patients with bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), pemphigoid gestationis (PG), pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Read More

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Pathological Relevance of Anti-Hsp70 IgG Autoantibodies in Epidermolysis Bullosa Acquisita.

Front Immunol 2022 20;13:877958. Epub 2022 Apr 20.

Department of Dermatology, Keck School of Medicine, University of Southern California, Los Angeles, CA, United States.

Stress-induced heat shock protein 70 (Hsp70) is a key intra- and extracellular molecular chaperone implicated in autoimmune processes. Highly immunogenic extracellular Hsp70 can activate innate and acquired (adaptive) immune responses driving the generation of anti-Hsp70 autoantibodies that are frequently observed in inflammatory/autoimmune disorders. We recently described the direct pathological role of extracellular Hsp70 in epidermolysis bullosa acquisita (EBA), an anti-type VII collagen autoantibody-mediated autoimmune blistering skin disease. Read More

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Case Report: Diagnostic and Therapeutic Challenges in Severe Mechanobullous Epidermolysis Bullosa Acquisita.

Front Immunol 2022 7;13:883967. Epub 2022 Apr 7.

Department of Dermatology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Collagen VII is the main constituent of the anchoring fibrils, important adhesive structures that attach the epidermis to the dermal extracellular matrix. Two disorders are caused by dysfunction of collagen VII, both characterized by skin and mucosa fragility, epidermolysis bullosa acquisita (EBA) and dystrophic epidermolysis bullosa (DEB). EBA and DEB share high clinical similarities with significant difference in patients' age of onset and pathogenesis. Read More

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Epidermolysis bullosa acquisita treated with ustekinumab: A case report.

SAGE Open Med Case Rep 2022 14;10:2050313X221091600. Epub 2022 Apr 14.

Division of Dermatology, McGill University Health Centre, Montreal General Hospital, Montreal, QC, Canada.

Epidermolysis bullosa acquisita is a rare autoimmune disease involving cutaneous blistering and scarring associated with collagen VII autoantibodies. Similarly, collagen VII autoantibodies are present in the majority of Crohn's disease patients and approximately a quarter of epidermolysis bullosa acquisita patients have coexisting Crohn's disease. Treatment options for epidermolysis bullosa acquisita are limited and are largely ineffective. Read More

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Regurgitation and prolapse of oesophageal mucosa: a dramatic presentation of the oesophagitis dissecans superficialis.

BMJ Case Rep 2022 Mar 22;15(3). Epub 2022 Mar 22.

Surgery, Jawaharlal Institute of Postgraduate Medical Education, Puducherry, Pondicherry, India

Oesophagitis dissecans superficialis is a rare benign entity that is usually self-limited, characterised by sloughing of the oesophageal mucosa. We preset a 38-year-old woman, known case of epidermolysis bullosa acquisita who presented to us with regurgitation and prolapse of the oesophageal mucosa from the mouth. Upper gastrointestinal endoscopy showed sloughing of the mucosa. Read More

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The Immunogenetics of Autoimmune Blistering Diseases.

Adv Exp Med Biol 2022 ;1367:173-212

Division of Dermatology, Rush University Medical Center, Chicago, USA.

Dermatological conditions constituting the group of autoimmune blistering diseases (AIBD) are characterized by loss of immunotolerance and humoral, as well as cellular, autoimmune responses that result in the development of bullae and erosions on the skin and mucous membranes. AIBDs are broadly categorized into pemphigus and pemphigoid classes with several distinct subtypes amongst them. Advances in genetics have allowed for the study and identification of alleles, and even single nucleotide polymorphisms, that harbor increased susceptibility or confer protection for the development of these conditions. Read More

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Epidermolysis bullosa aquisita following immune-checkpoint inhibitor treatment for metastatic melanoma.

Clin Exp Dermatol 2022 Jun 26;47(6):1198-1199. Epub 2022 Apr 26.

St John's Institute of Dermatology, Guy's Hospital, Guy's & St Thomas' NHS Foundation Trust, London, UK.

A case of epidermolysis bullosa aquisita following immunotherapy for melanoma. This adds to the repertoire of subepidermal blistering disorders documented following immune checkpoint therapy. Read More

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Epidermolysis bullosa acquisita: a case series of three paediatric patients.

Clin Exp Dermatol 2022 Jan 26. Epub 2022 Jan 26.

Department of Dermatology, Great Ormond Street Hospital NHS Foundation Trust, London, UK.

Epidermolysis bullosa acquisita is a highly uncommon condition in the paediatric population. This article describes three children with this disease, different clinical presentation and management. It also reviews the most relevant articles on this topic. Read More

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January 2022

Brunsting-Perry pemphigoid: a systematic review.

Int J Dermatol 2022 Jan 20. Epub 2022 Jan 20.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Background: Brunsting-Perry pemphigoid (BPP) is a rare, autoimmune bullous skin disorder classified within the spectrum of mucous membrane pemphigoid (MMP).

Materials And Methods: An a priori protocol was designed based on PRISMA guidelines. PubMed and Scopus databases were searched for English-language articles concerning BPP published between 1950 and July 2021. Read More

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January 2022

Subepidermal type VII collagen speckles as an additional clue for diagnosing epidermolysis bullosa acquisita by salt-split skin serum analysis.

J Eur Acad Dermatol Venereol 2022 05 2;36(5):e384-e386. Epub 2022 Feb 2.

Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

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C5aR2 deficiency ameliorates inflammation in murine epidermolysis bullosa acquisita by regulating FcγRIIb expression on neutrophils.

J Invest Dermatol 2022 Jan 7. Epub 2022 Jan 7.

Institute for Systemic Inflammation Research (ISEF), University of Lübeck, Lübeck, Germany. Electronic address:

Epidermolysis bullosa acquisita (EBA) is a rare blistering skin disease induced by autoantibodies directed against type VII collagen (COL7). Transfer of antibodies against murine COL7 (mCOL7) into mice mimics the effector phase of EBA and results in a subepidermal blistering phenotype. Activation of the complement system, and especially the C5a/C5aR1 axis driving neutrophil activation, are critical for EBA pathogenesis. Read More

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January 2022

The pathogeneses of pemphigus and pemphigoid diseases.

J Dermatol Sci 2021 Dec;104(3):154-163

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

Autoimmune bullous diseases (AIBDs) are skin disorders which are mainly induced by autoantibodies against desmosomal or hemidesmosomal structural proteins. Previous studies using patients' samples and animal disease models identified target antigens and elucidated the mechanisms of blister formation. Pemphigus has been the subject of more active clinical and basic research than any other AIBD. Read More

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December 2021

Natural Occurrence of Autoantibodies against Basement Membrane Proteins in Epidermolysis Bullosa.

J Invest Dermatol 2022 Jul 26;142(7):2014-2019.e3. Epub 2021 Nov 26.

Center for Blistering Diseases, European Reference Network-Skin Reference Center (ERN-Skin), University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Electronic address:

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Nutrition and bullous diseases.

Clin Dermatol 2022 Mar-Apr;40(2):156-165. Epub 2021 Nov 19.

Department of Dermatology, University of Connecticut Health Center, Farmington, Connecticut, USA. Electronic address:

Although relatively uncommon, autoimmune bullous diseases carry the risk of increased mortality and can significantly impact quality of life. This group of diseases is broad and encompasses subepidermal conditions such as bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, and linear IgA bullous dermatosis, as well as intraepidermal conditions such as pemphigus and its variants. The pathophysiology of each condition is incompletely understood but broadly involves the formation of autoantibodies targeting skin adhesion proteins, a process that relies on a complex interplay between a dysregulated immune system, genetic predisposition, and environmental factors. Read More

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Multiple Modes of Action Mediate the Therapeutic Effect of Intravenous IgG in Experimental Epidermolysis Bullosa Acquisita.

J Invest Dermatol 2022 Jun 15;142(6):1552-1564.e8. Epub 2021 Nov 15.

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

Substitution of IgG in antibody deficiency or application of high-dose intravenous IgG in patients with autoimmunity is a well-established treatment. However, data on the mode of action of intravenous IgG are controversial and may differ for distinct diseases. In this study, we investigated the impact and molecular mechanism of high-dose IgG (hd-IgG) treatment in murine autoantibody‒induced skin inflammation, namely, epidermolysis bullosa acquisita. Read More

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Evidence for a role of extracellular heat shock protein 70 in epidermolysis bullosa acquisita.

Exp Dermatol 2022 04 12;31(4):528-534. Epub 2021 Nov 12.

Department of Dermatology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.

Heat shock protein 90 (Hsp90) and Hsp70 are chaperones implicated in different inflammatory disorders, given their property to impact innate and adaptive immune responses. Here, we determined the so far unknown role of extracellular Hsp70 in epidermolysis bullosa acquisita (EBA), an anti-type VII collagen autoantibody-mediated blistering dermatosis. The in vivo pathophysiological relevance of extracellular Hsp70 was demonstrated in an anti-type VII collagen antibody transfer-induced EBA mouse model in which elevated blood levels of this chaperone were recorded. Read More

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Preventive but Not Therapeutic Topical Application of Local Anesthetics Can Inhibit Experimental Epidermolysis Bullosa Acquisita in Mice.

Front Immunol 2021 12;12:750160. Epub 2021 Oct 12.

Priority Area Asthma & Allergy, Research Center Borstel, Airway Research Center North (ARCN), Member of the German Center for Lung Research Deutsches Zentrum für Lungenforschung (DZL), Borstel, Germany.

Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disorder characterized and caused by autoantibodies against type VII collagen (COL7). Although it has been noticed that EBA in both patients and mice is associated with an increased scratching, it is not clear whether and how the scratching contributes to disease manifestation. Hence, we here aimed to validate this clinical observation and also to investigate the potential contribution of increased scratching in EBA pathogenesis in mice. Read More

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January 2022

Bullous Pemphigoid and Other Pemphigoid Dermatoses.

Medicina (Kaunas) 2021 Oct 4;57(10). Epub 2021 Oct 4.

Department of Dermatology, College of Medicine, University of Florida, Gainesville, FL 32606, USA.

The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous pemphigoid. Its variable clinical presentation, with or without frank bullae, is linked by significant pruritus afflicting the elderly. Read More

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October 2021

Phospholipase Cγ2 Is Essential for Experimental Models of Epidermolysis Bullosa Acquisita.

J Invest Dermatol 2022 04 14;142(4):1114-1125. Epub 2021 Oct 14.

Department of Physiology, Faculty of Medicine, Semmelweis University, Budapest, Hungary. Electronic address:

Phospholipase Cγ2 (PLCγ2) mediates tyrosine kinase‒coupled receptor signaling in various hematopoietic lineages. Although PLCγ2 has been implicated in certain human and mouse inflammatory disorders, its contribution to autoimmune and inflammatory skin diseases is poorly understood. In this study, we tested the role of PLCγ2 in a mouse model of epidermolysis bullosa acquisita triggered by antibodies against type VII collagen (C7), a component of the dermo-epidermal junction. Read More

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A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey.

Australas J Dermatol 2021 Nov 12;62(4):e496-e503. Epub 2021 Oct 12.

Faculty of Medicine, Department of Dermatology, Karadeniz Technical University, Trabzon, Turkey.

Background: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. Read More

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November 2021

Topical Application of the PI3Kβ-Selective Small Molecule Inhibitor TGX-221 Is an Effective Treatment Option for Experimental Epidermolysis Bullosa Acquisita.

Front Med (Lausanne) 2021 7;8:713312. Epub 2021 Sep 7.

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

Class I phosphoinositide 3-kinases (PI3K) have been implemented in pathogenesis of experimental epidermolysis bullosa acquisita (EBA), an autoimmune skin disease caused by type VII collagen (COL7) autoantibodies. Mechanistically, inhibition of specific PI3K isoforms, namely PI3Kβ or PI3Kδ, impaired immune complex (IC)-induced neutrophil activation, a key prerequisite for EBA pathogenesis. Data unrelated to EBA showed that neutrophil activation is also modulated by PI3Kα and γ, but their impact on the EBA has, so far, remained elusive. Read More

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September 2021

Serration pattern analysis as a practical adjunct tool for categorization of subepidermal autoimmune blistering diseases.

Indian J Dermatol Venereol Leprol 2021 Nov-Dec;87(6):778-786

Department of Dermatology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India.

Background: Serration pattern analysis helps in the classification of subepidermal autoimmune blistering disorders; more precisely, it helps to differentiate epidermolysis bullosa acquisita from other subepidermal autoimmune blistering disorders. Most of the published reports of this tool have come from a single center.

Objectives: The objectives of the study were to study the utility of serration pattern analysis in classifying subepidermal autoimmune blistering disorders. Read More

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February 2022

Pediatric epidermolysis bullosa acquisita: A review.

Pediatr Dermatol 2021 Sep 2;38(5):1047-1050. Epub 2021 Aug 2.

Department of Dermatology, University of Central Florida College of Medicine, Orlando, FL, USA.

Epidermolysis bullosa acquisita (EBA) is an acquired autoimmune blistering skin disorder that is rare in adults and even rarer in childhood. This review aims to identify cases of pediatric EBA and report their clinical features and course. Our literature review was conducted in MEDLINE using the search terms related to juvenile epidermolysis bullosa acquisita. Read More

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September 2021