973 results match your criteria Epidermolysis Bullosa Acquisita


Dapsone Suppresses Disease in Preclinical Murine Models of Pemphigoid Diseases.

J Invest Dermatol 2021 May 22. Epub 2021 May 22.

Department of Dermatology, Allergy, and Venereology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany; Center for Research on Inflammation of the Skin (CRIS), University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany. Electronic address:

Epidermolysis bullosa acquisita (EBA) and mucous membrane pemphigoid (MMP) are autoimmune blistering diseases characterized by mucocutaneous blisters elicited by an autoantibody-mediated immune response against specific proteins of the epidermal basement membrane. The antibiotic dapsone is frequently used to treat both diseases, but its therapeutic effectiveness is uncertain, and its mode of action in these diseases is largely unknown. We evaluated the effect of dapsone in antibody transfer mouse models of EBA and MMP, which do not allow drawing conclusions on clinical treatment regimens but can be instrumental to partially uncover the mode(s) of action of dapsone in these diseases. Read More

View Article and Full-Text PDF

Immunobullous disease.

Clin Med (Lond) 2021 05;21(3):162-165

St John's Institute of Dermatology, London, UK

Immunobullous diseases are blistering cutaneous disorders that are caused by pathogenic antibodies binding to protein targets within the skin. There are a range of immunobullous disorders with characteristic morphology that relates to the structural properties of the target protein. In this article we will describe the pathogenesis, clinical features and treatment of the most common immunobullous disorders. Read More

View Article and Full-Text PDF

Observational Study of a Cohort of Patients Treated in a National Referral Center.

Actas Dermosifiliogr (Engl Ed) 2021 May 10. Epub 2021 May 10.

Servicio de Dermatología, Hospital Universitario La Paz, Madrid, Spain.

Background And Objective: Epidermolysis bullosa (EB) is a heterogeneous group of inherited disorders characterized by a high degree of mucocutaneous fragility. This study aimed to describe the clinical and epidemiologic characteristics of patients with EB treated in Hospital Universitario La Paz, a national referral center for inherited EB.

Material And Methods: Observational, retrospective, single-center study. Read More

View Article and Full-Text PDF

Epidermolysis bullosa acquisita: an uncommon cause of esophageal stricture.

Oxf Med Case Reports 2021 Apr 28;2021(4):omab010. Epub 2021 Apr 28.

Division of Gastroenterology and Hepatology, Montefiore Medical Center, Bronx, NY, USA.

Epidermolysis bullosa acquisita (EBA) encompasses a wide spectrum of rare diseases with a common genetic origin transmitted in an autosomal recessive fashion. Mild forms of non-inflammatory EBA are characterized by skin lesions and have gained great relevance in the literature. However, resistant inflammatory EBA with widespread mucosal involvement remains a rare entity given its low prevalence. Read More

View Article and Full-Text PDF

The development of mucous membrane epidermolysis bullosa acquisita in a pediatric patient.

JAAD Case Rep 2021 May 10;11:3-5. Epub 2021 Mar 10.

University of Nebraska Medical Center, Department of Dermatology, Omaha, Nebraska.

View Article and Full-Text PDF

In vitro diagnostics for the medical dermatologist. Part I: Autoimmune tests.

J Am Acad Dermatol 2021 Apr 20. Epub 2021 Apr 20.

Division of Dermatology, Department of Internal Medicine, The Ohio State University, Columbus, Ohio. Electronic address:

Despite the expansion of available in vitro laboratory tests at a rate far exceeding that of dermatologic pharmaceuticals, the existing literature is dominated by discussion of the latter. With the advent of numerous new tests, it can be difficult for practicing dermatologists to stay up-to-date on the available options, methodologies, and recommendations for when to order one test over another. Understanding the inherent strengths and weaknesses of these options is necessary to inform appropriate ordering and proper interpretation of the results. Read More

View Article and Full-Text PDF

Diagnosis of Epidermolysis Bullosa Acquisita: Multicentre Comparison of Different Assays for Serum Anti-type VII Collagen Reactivity.

Acta Derm Venereol 2021 Mar 23;101(3):adv00420. Epub 2021 Mar 23.

Department of Dermatology, University of Lubeck, DE-23522 Lubeck, Germany.

Epidermolysis bullosa acquisita is a pemphigoid disease characterized by autoantibodies against type VII collagen. This study compared the sensitivity and specificity of 6 diagnostic assays: type VII collagen non-collagenous domains enzyme-linked immunoassay (NC1/2 ELISA) (MBL, Nagoya, Japan); type VII collagen NC1 ELISA (Euroimmun, Lübeck, Germany); indirect immunofluorescence (IF) microscopy test based on the expression of recombinant NC1 in a human cell line (NC1 BIOCHIP®; Euroimmun); full-length recombinant type VII collagen ELISA; immunoblotting with full-length type VII collagen in the extract of human dermis; and immunoblotting with recombinant NC1. Immunoblotting with recombinant NC1 showed a sensitivity of 93. Read More

View Article and Full-Text PDF

Subepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.

J Am Acad Dermatol 2021 Jul 5;85(1):1-14. Epub 2021 Mar 5.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.

Subepithelial autoimmune blistering dermatoses are a group of rare skin disorders that are characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The third article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major subepithelial autoimmune blistering dermatoses, including bullous pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, mucous membrane pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis. Read More

View Article and Full-Text PDF

Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.

J Am Acad Dermatol 2021 Jul 5;85(1):18-27. Epub 2021 Mar 5.

Epiphany Dermatology, Dallas, Texas. Electronic address:

Subepidermal (subepithelial) autoimmune blistering dermatoses are a group of rare skin disorders characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The fourth article in this continuing medical education series presents the current validated disease activity scoring systems, serologic parameters, treatments, and clinical trials for bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, anti-p200 pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis. Read More

View Article and Full-Text PDF

An atypical case of pediatric epidermolysis bullosa acquisita: Review of diagnosis and pitfalls.

JAAD Case Rep 2021 Mar 20;9:81-85. Epub 2021 Jan 20.

Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin.

View Article and Full-Text PDF

Inhibition of Glucose Metabolism Abrogates the Effector Phase of Bullous Pemphigoid-Like Epidermolysis Bullosa Acquisita.

J Invest Dermatol 2021 Feb 18. Epub 2021 Feb 18.

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:

Bullous pemphigoid-like epidermolysis bullosa acquisita (EBA) is an autoantibody-driven, granulocyte-mediated skin disease. The role of cellular metabolism and its potential as a therapeutic target in EBA are unknown. We investigated the effect of 2-deoxy-D-glucose and metformin in the antibody transfer model of EBA. Read More

View Article and Full-Text PDF
February 2021

A Mitochondrial Polymorphism Alters Immune Cell Metabolism and Protects Mice from Skin Inflammation.

Int J Mol Sci 2021 Jan 20;22(3). Epub 2021 Jan 20.

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

Several genetic variants in the mitochondrial genome (mtDNA), including ancient polymorphisms, are associated with chronic inflammatory conditions, but investigating the functional consequences of such mtDNA polymorphisms in humans is challenging due to the influence of many other polymorphisms in both mtDNA and the nuclear genome (nDNA). Here, using the conplastic mouse strain B6-mt, we show that in mice, a maternally inherited natural mutation (m.7778G > T) in the mitochondrially encoded gene ATP synthase 8 () of complex V impacts on the cellular metabolic profile and effector functions of CD4 T cells and induces mild changes in oxidative phosphorylation (OXPHOS) complex activities. Read More

View Article and Full-Text PDF
January 2021

Diagnostic Value and Practicability of Serration Pattern Analysis by Direct Immunofluorescence Microscopy in Pemphigoid Diseases.

Acta Derm Venereol 2021 Mar 9;101(3):adv00410. Epub 2021 Mar 9.

Department of Dermatology, University of Lubeck, DE-23522 Lubeck, Germany.

In pemphigoid diseases, direct immunofluorescence can be used to differentiate 2 patterns of antibody deposition at the dermal-epidermal junction; u- and n-serrated pattern. The u-serrated pattern is found in epidermolysis bullosa acquisita, and n-serrated pattern in all other pemphigoid diseases. To determine the detection frequency of these serrated patterns and the optimal thickness of biopsy cryosections, 2 patient cohorts obtained form our routine autoimmune laboratory were analysed; a retrospective cohort (n = 226) and a prospective cohort (n = 156). Read More

View Article and Full-Text PDF

Dermatological Manifestations in Inflammatory Bowel Diseases.

J Clin Med 2021 Jan 19;10(2). Epub 2021 Jan 19.

Gastroenterology Unit, Department of Biomedical and Clinical Sciences, "L.Sacco" Hospital, 20157 Milano, Italy.

Inflammatory bowel diseases (IBDs) may be associated with extra-intestinal manifestations. Among these, mucocutaneous manifestations are relatively frequent, often difficult to diagnose and treat, and may complicate the course of the underlying disease. In the present review, a summary of the most relevant literature on the dermatologic manifestations occurring in patients with inflammatory bowel diseases has been reviewed. Read More

View Article and Full-Text PDF
January 2021

Epidermolysis bullosa acquisita as an adverse effect from rituximab therapy: A case report.

Medicine (Baltimore) 2020 Dec;99(49):e23496

The People's Hospital of Huantai County, Zibo, Shandong, China.

Rationale: Rituximab is a monoclonal antibody directed against B cells and is a first-line agent for the treatment of B cell lymphoma and a second-line agent for the treatment of idiopathic thrombocytopenic purpura (ITP). It has also been used for the treatment of several other autoimmune diseases. Epidermolysis bullosa acquisita (EBA) has never been reported as an adverse effect resulted from rituximab therapy. Read More

View Article and Full-Text PDF
December 2020

Propranolol Off-Target: A New Therapeutic Option in Neutrophil-Dependent Dermatoses?

J Invest Dermatol 2020 12;140(12):2326-2329

Department of Dermatology and Allergology, Philipps-Universität Marburg, Marburg, Germany. Electronic address:

Epidermolysis bullosa acquisita (EBA) is a rare subepidermal blistering dermatosis characterized by autoantibodies targeting collagen VII (COL7), an essential component of the anchoring fibrils, located in the sublamina densa of the dermal‒epidermal junction. In EBA, tissue-bound autoantibodies cause the recruitment and subsequent activation of neutrophils, which eventually lead to subepidermal blistering through the release of proteases and ROS. Thus, targeting either pathogenic IgG autoantibodies or neutrophil recruitment or activation has shown efficacy in experimental murine EBA models and patients with EBA. Read More

View Article and Full-Text PDF
December 2020

European dermatology forum - updated guidelines on the use of extracorporeal photopheresis 2020 - part 1.

J Eur Acad Dermatol Venereol 2020 Dec 6;34(12):2693-2716. Epub 2020 Oct 6.

Division of Haematology, LKH-Univ. Klinikum Graz, Medical University of Graz, Graz, Austria.

Background: Following the first investigational study on the use of extracorporeal photopheresis for the treatment of cutaneous T-cell lymphoma published in 1983, this technology has received continued use and further recognition for additional earlier as well as refractory forms. After the publication of the first guidelines for this technology in the JEADV in 2014, this technology has maintained additional promise in the treatment of other severe and refractory conditions in a multi-disciplinary setting. It has confirmed recognition in well-known documented conditions such as graft-versus-host disease after allogeneic bone marrow transplantation, systemic sclerosis, solid organ transplant rejection including lung, heart and liver and to a lesser extent inflammatory bowel disease. Read More

View Article and Full-Text PDF
December 2020

Diagnosis of autoimmune subepidermal bullous diseases with mucous membrane involvement based on laser-scanning confocal microscopy.

Eur J Dermatol 2020 Oct;30(5):516-523

Department of Dermatology and Immunodermatology, Medical University of Warsaw, Poland.

Background: Mucosal involvement in autoimmune subepidermal blistering disorders (ASBD) may represent the only or predominant localization. Circulating autoantibodies are detected in 50% cases.

Objective: The aim of this study was to evaluate the usefulness of fluorescence overlay antigen mapping by laser-scanning confocal microscopy (FOAM-LSCM) to identify ASBD with mucosal involvement in oral mucosa specimens. Read More

View Article and Full-Text PDF
October 2020

BIOCHIP mosaic for the diagnosis of autoimmune bullous diseases in Chinese patients.

Eur J Dermatol 2020 Aug;30(4):338-344

Department of Dermatology, Peking University First Hospital, and National Clinical Research Center for Skin and Immune Diseases, and Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China.

Background: Autoimmune blistering diseases (AIBDs) are a group of fatal diseases with specific autoantibodies. BIOCHIP mosaic is a novel and all-in-one measure used for the rapid diagnosis of AIBDs.

Objectives: To evaluate the diagnostic accuracy based on BIOCHIP mosaic (FA1501-1005-60) in Chinese patients with AIBDs. Read More

View Article and Full-Text PDF

European dermatology forum: Updated guidelines on the use of extracorporeal photopheresis 2020 - Part 2.

J Eur Acad Dermatol Venereol 2021 Jan 22;35(1):27-49. Epub 2020 Sep 22.

LKH-Univ. Klinikum Graz, Division of Haematology, Medical University of Graz, Graz, Austria.

Background: Following the first investigational study on the use of extracorporeal photopheresis for the treatment of cutaneous T-cell lymphoma published in 1983, this technology has received continued use and further recognition for additional earlier as well as refractory forms. After the publication of the first guidelines for this technology in the JEADV in 2014, this technology has maintained additional promise in the treatment of other severe and refractory conditions in a multidisciplinary setting. It has confirmed recognition in well-known documented conditions such as graft-vs. Read More

View Article and Full-Text PDF
January 2021

The G Protein-Coupled Receptor (GPR) 15 Counteracts Antibody-Mediated Skin Inflammation.

Front Immunol 2020 14;11:1858. Epub 2020 Aug 14.

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

The G protein-coupled receptor 15 (GPR15) has recently been highlighted as an important regulator of T cell trafficking into the gut under physiological and pathophysiological conditions. Additionally, circumstantial evidence has accumulated that GPR15 may also play a role in the regulation of chronic inflammation. However, the (patho)physiological significance of GPR15 has, in general, remained rather enigmatic. Read More

View Article and Full-Text PDF

Epidermolysis Bullosa Acquisita Mimicking Linear IgA Bullous Disease in a 5-year-old Child.

Acta Dermatovenerol Croat 2020 Aug;28(2):109-112

Karen Manuelyan, MD, PhD, "Prof. St. Kirkovich" University Hospital, 2 General Stoletov Str., Stara Zagora, Bulgaria;

We present a case of a 5-year-old child with epidermolysis bullosa acquisita, clinically resembling linear IgA bullous disease. The case demonstrates that autoimmune bullous dermatoses in childhood may show a clinical overlap, which makes the diagnosis based on clinical features highly unreliable. Specific immunofluorescence and immunoserological tests are crucial for precise diagnosis - in our case circulating antibodies against collagen VII were detected using ELISA and indirect immunofluorescence on transfected cells. Read More

View Article and Full-Text PDF

Dermatological Manifestations in Pediatric Inflammatory Bowel Disease.

Medicina (Kaunas) 2020 Aug 23;56(9). Epub 2020 Aug 23.

Department of Dermatology, "Carol Davila" University of Medicine and Pharmacy, 050474 Bucharest, Romania.

Background and Objectives Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated with numerous extraintestinal manifestations, making its management a real challenge for the pediatric gastroenterologist. Dermatological manifestations in IBD are either specific, related to the disease activity or treatment-associated, or non-specific. This literature review aims to identify and report the dermatological manifestations of IBD in children, the correlation between their appearance and the demographical characteristics, the relationship between these lesions and disease activity, and to highlight the impact of dermatological manifestations on an IBD treatment regime. Read More

View Article and Full-Text PDF

12/15-Lipoxygenase choreographs the resolution of IgG-mediated skin inflammation.

J Autoimmun 2020 12 4;115:102528. Epub 2020 Aug 4.

Department of Dermatology, Allergy, and Venereology, University of Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Germany; Center for Research on Inflammation of the Skin (CRIS), University of Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Germany. Electronic address:

Autoimmune diseases are defined by an immune response against a specific autoantigen, driven by antigen-specific T cells or antibodies. While the mechanisms resolving brief episodes of acute inflammation elicited by microbial components or tissue injury are well understood, the mechanisms resolving tissue inflammation in autoimmune diseases are still largely elusive. We have, therefore, addressed the mechanisms of resolution in IgG-mediated autoimmune diseases using a mouse model of the pemphigoid disease "bullous pemphigoid-like epidermolysis bullosa acquisita" (BP-like EBA) as prototypical example. Read More

View Article and Full-Text PDF
December 2020

IgG Fc N-Glycosylation Translates MHCII Haplotype into Autoimmune Skin Disease.

J Invest Dermatol 2021 Feb 10;141(2):285-294. Epub 2020 Jul 10.

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

The major histocompatibility complex haplotype represents the most prevalent genetic risk factor for the development of autoimmune diseases. However, the mechanisms by which major histocompatibility complex-associated genetic susceptibility translates into autoimmune disease are not fully understood. Epidermolysis bullosa acquisita is an autoimmune skin-blistering disease driven by autoantibodies to type VII collagen. Read More

View Article and Full-Text PDF
February 2021

Multiple milia formation in blistering diseases.

Int J Womens Dermatol 2020 Jun 1;6(3):199-202. Epub 2020 Apr 1.

Department of Dermatology, University of New South Wales, Sydney, Australia.

Background: Milia are superficial keratinous cysts seen as pearly white, dome-shaped lesions 1-2 mm in diameter. Milia are associated with diseases that cause subepidermal blistering, such as hereditary forms of epidermolysis bullosa, epidermolysis bullosa acquisita, bullous pemphigoid, bullous lichen planus, and porphyria cutanea tarda. Multiple eruptive milia are rare and more extensive in number than primary milia. Read More

View Article and Full-Text PDF

Gastrointestinal involvement of primary skin diseases.

J Eur Acad Dermatol Venereol 2020 Dec 25;34(12):2766-2774. Epub 2020 Jun 25.

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

View Article and Full-Text PDF
December 2020

Propranolol Is an Effective Topical and Systemic Treatment Option for Experimental Epidermolysis Bullosa Acquisita.

J Invest Dermatol 2020 12 22;140(12):2408-2420. Epub 2020 May 22.

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

Propranolol is an 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 ROS after immune complex stimulation. Read More

View Article and Full-Text PDF
December 2020

Tense bullae on the hands.

Cutis 2020 Mar;105(3):E9-E10

Department of Internal Medicine, Division of Dermatology, University of Kansas Medical Center, Kansas City, USA.

View Article and Full-Text PDF