899 results match your criteria Epidermolysis Bullosa Acquisita


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

Ophthalmol Ther 2019 Jan 29. 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
January 2019
1 Read

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
1 Read

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
9 Reads

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

J Dermatol 2019 Jan 23. 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
January 2019
3 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
3 Reads

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

Int J Med Inform 2019 Feb 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

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

J Immunol 2018 Dec 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
1 Read

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
14 Reads

Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients.

Skin Appendage Disord 2018 Oct 20;4(4):308-311. Epub 2018 Apr 20.

Universidade Federal Fluminense, Niterói, Brazil.

Brunsting-Perry type pemphigoid (BPP) is a rare subepidermal blistering disease and a cause of secondary cicatricial alopecia. It was originally described by Brunsting and Perry in 1957 as a rare variant of cicatricial pemphigoid, characterized by bullous lesions limited to the head, neck, scalp, and upper trunk with mild or no mucosal involvement. We report 2 cases of BPP cicatricial alopecia with histopathology of subepidermal blister formation, different clinical presentation, and different salt-split test results. Read More

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http://dx.doi.org/10.1159/000485570DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6219224PMC
October 2018
18 Reads

Bullous autoimmune dermatoses.

J Dtsch Dermatol Ges 2018 Nov;16(11):1339-1358

Department of Dermatology and Allergology, Marburg University Medical Center, Marburg, Germany.

Pathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in blister formation. Depending on the respective target proteins of the autoimmune response and their location in the skin, a distinction is made between intraepidermal (pemphigus disorders), junctional (pemphigoid disorders), and subepidermal (epidermolysis bullosa acquisita, dermatitis herpetiformis) autoimmune blistering diseases. The most common bullous autoimmune dermatosis, bullous pemphigoid is characterized by marked clinical variability and intense pruritus. Read More

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http://dx.doi.org/10.1111/ddg.13688DOI Listing
November 2018
16 Reads

Post-orf epidermolysis bullosa acquisita.

J Eur Acad Dermatol Venereol 2018 Oct 25. Epub 2018 Oct 25.

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

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http://doi.wiley.com/10.1111/jdv.15299
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http://dx.doi.org/10.1111/jdv.15299DOI Listing
October 2018
10 Reads

In utero development of epidermolysis bullosa acquisita.

Pediatr Dermatol 2019 Jan 18;36(1):e46-e47. Epub 2018 Oct 18.

Department of Dermatology, Vanderbilt University Medical Center, Nashville, Tennessee.

We report the case of an infant born with perioral vesicles that rapidly spread to involve his mouth and the majority of his body. Histopathology, immunofluorescence, and enzyme-linked immunohistochemistry assays confirmed a diagnosis of epidermolysis bullosa acquisita (EBA). His mother had no history of EBA, and serum indirect immunofluorescence was negative. Read More

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http://doi.wiley.com/10.1111/pde.13704
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http://dx.doi.org/10.1111/pde.13704DOI Listing
January 2019
6 Reads

Detection of anti-type VII collagen IgE antibodies in epidermolysis bullosa acquisita.

Br J Dermatol 2018 Oct 11. Epub 2018 Oct 11.

Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan.

Background: Epidermolysis bullosa acquisita (EBA) is a rare pemphigoid disease involving autoantibodies to type VII collagen (COL7), a major structural component of anchoring fibrils. IgE autoantibodies to type XVII collagen (BP180) have been identified in bullous pemphigoid (BP), the prototype of pemphigoid diseases. Although the pathogenic relevance of IgG anti-COL7 has been investigated, that of IgE in EBA remains unclear. Read More

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http://doi.wiley.com/10.1111/bjd.17310
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http://dx.doi.org/10.1111/bjd.17310DOI Listing
October 2018
1 Read

The use of rituximab in treatment of epidermolysis bullosa acquisita: Three new cases and a review of the literature.

Dermatol Ther 2018 11 3;31(6):e12726. Epub 2018 Oct 3.

Department of Medicine, Health Sciences Campus at Lake Nona, Orlando, FL.

Epidermolysis bullosa acquisita (EBA) is a rare, subepidermal blistering disease affecting the skin and mucous membranes that often remains refractory to standard immunosuppressive therapy. We present three original cases and a review of the literature of 20 cases of refractory EBA treated with rituximab as monotherapy or in combination with other agents. Complete control (with or without therapy) and remission were seen in 56% of patients treated with rituximab monotherapy and 75% of patients treated with rituximab and immunoadsorption (IA). Read More

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http://dx.doi.org/10.1111/dth.12726DOI Listing
November 2018
5 Reads

Accessible Diagnostic Methods to Differentiate between Epidermolysis Bullosa Acquisita and Other Subepidermal Autoimmune Bullous Diseases.

Authors:
Khalaf Kridin

Indian J Dermatol 2018 Sep-Oct;63(5):445-448

Department of Dermatology, Rambam Health Care Campus, Haifa, Israel. E-mail:

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http://dx.doi.org/10.4103/ijd.IJD_75_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6124243PMC
September 2018
3 Reads

Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients.

Orphanet J Rare Dis 2018 Sep 4;13(1):153. Epub 2018 Sep 4.

Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, D-23538, Lübeck, Germany.

Background: Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Several clinical phenotypes have been described, but subepidermal blistering is characteristic of all variants. Limited data on clinical and immunopathological characteristics and treatment outcomes in EBA are available. Read More

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http://dx.doi.org/10.1186/s13023-018-0896-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6122731PMC
September 2018
2 Reads

Direct Immunofluorescence of Mechanobullous Epidermolysis Bullosa Acquisita, Porphyria Cutanea Tarda and Pseudoporphyria.

Acta Derm Venereol 2019 01;99(1):26-32

Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, Hanzeplein 1, NL-9700 RB Groningen, The Netherlands.

Mechanobullous epidermolysis bullosa acquisita (mEBA) can have a clinical presentation that is very similar to other blistering diseases, such as porphyria cutanea tarda (PCT) and pseudoporphyria. Direct immunofluorescence is an important feature in the diagnosis of mEBA, although features that overlap with PCT and pseudoporphyria have been reported. This retrospective observational study investigated whether direct immunofluorescence can discriminate mEBA from PCT and pseudoporphyria. Read More

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http://dx.doi.org/10.2340/00015555-3021DOI Listing
January 2019
4 Reads

The International Bullous Diseases Group consensus on diagnostic criteria for epidermolysis bullosa acquisita: a useful tool for dermatologists.

Authors:
J Yamagami

Br J Dermatol 2018 Jul;179(1)

Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo, 160-8582, Japan.

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http://dx.doi.org/10.1111/bjd.16440DOI Listing
July 2018
2 Reads

The Immunometabolomic Interface Receptor Hydroxycarboxylic Acid Receptor 2 Mediates the Therapeutic Effects of Dimethyl Fumarate in Autoantibody-Induced Skin Inflammation.

Front Immunol 2018 14;9:1890. Epub 2018 Aug 14.

Institute for Experimental and Clinical Pharmacology and Toxicology, University of Lübeck, Lübeck, Germany.

The drug dimethyl fumarate (DMF) is in clinical use for the treatment of psoriasis and multiple sclerosis. In addition, it has recently been demonstrated to ameliorate skin pathology in mouse models of pemphigoid diseases, a group of autoimmune blistering diseases of the skin and mucous membranes. However, the mode of action of DMF in inflammatory skin diseases has remained elusive. Read More

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http://dx.doi.org/10.3389/fimmu.2018.01890DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6102353PMC
August 2018
4 Reads

Epidermolysis bullosa acquisita in association with mantle cell lymphoma.

Cutis 2018 06;101(6):E13-E15

DermDOX Center for Dermatology, Hazleton, Pennsylvania, USA.

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June 2018
4 Reads

Therapeutic Effect of a Novel Phosphatidylinositol-3-Kinase δ Inhibitor in Experimental Epidermolysis Bullosa Acquisita.

Front Immunol 2018 12;9:1558. Epub 2018 Jul 12.

Skin Biology and Pharmacology, Almirall R&D, Barcelona, Spain.

Epidermolysis bullosa acquisita (EBA) is a rare, but prototypical, organ-specific autoimmune disease, characterized and caused by autoantibodies against type VII collagen (COL7). Mucocutaneous inflammation, blistering, and scarring are the clinical hallmarks of the disease. Treatment of EBA is difficult and mainly relies on general immunosuppression. Read More

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http://dx.doi.org/10.3389/fimmu.2018.01558DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6052048PMC
July 2018
7 Reads

The Value of Type IV Collagen Immunohistochemical Staining in the Differential Diagnosis of Autoimmune Subepidermal Bullous Diseases.

Acta Dermatovenerol Croat 2018 Jun;26(2):133-138

Hai-Jin Park, MD, Inje Univ. Ilsan Paik Hospital, 170 Juwha-Ro, Ilsanseo-gu, Goyang, Gyeonggi-do, Korea;

Autoimmune subepidermal bullous diseases (AISBDs) exhibit various clinical presentations, histological appearances, prognoses, and responses to treatment. Many diagnostic techniques, such as direct immunofluorescence (IF), indirect salt-split skin IF, and enzyme-linked immunosorbent assays, are used in the differential diagnoses of AISBDs. However, these techniques require fresh frozen tissue, expensive laboratory equipment, and sophisticated laboratory techniques. Read More

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June 2018
6 Reads

Diagnosis of anti-laminin γ-1 pemphigoid by immunoblot analysis.

J Eur Acad Dermatol Venereol 2018 Jul 4. Epub 2018 Jul 4.

Department of Dermatology and Allergology, Philipps-University, Marburg, Germany.

Background: Anti-laminin-γ1 (lam-γ1) pemphigoid, a recently described immunobullous disorder sharing immune serological features of bullous pemphigoid and epidermolysis bullosa acquisita (EBA), is characterized by the detection of serum IgG autoantibodies against the lam-γ1 chain, a 200 kDa heterotrimeric component of the dermal-epidermal junction (DEJ).

Objective: The aim of the study was to develop an easy-to-perform and reliable assay for the serological detection of anti-lam-γ1 IgG autoantibodies. The clinical appearance alone is not sufficient to establish diagnosis of anti-lam-γ1 pemphigoid and rather requires immune serological evidence of (i) IgG reactivity against the dermal portion of salt-split human skin; (ii) exclusion of IgG against other components of the DEJ; and (iii) IgG reactivity with a 200 kDa protein of dermal extracts by immunoblot analysis (IB). Read More

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http://doi.wiley.com/10.1111/jdv.15170
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http://dx.doi.org/10.1111/jdv.15170DOI Listing
July 2018
9 Reads

Granzyme B is elevated in autoimmune blistering diseases and cleaves key anchoring proteins of the dermal-epidermal junction.

Sci Rep 2018 Jun 26;8(1):9690. Epub 2018 Jun 26.

International Collaboration On Repair Discoveries (ICORD) Research Centre, Vancouver, BC, V5Z 1M9, Canada.

In healthy skin, epidermis and dermis are anchored together at the dermal-epidermal junction (DEJ), a specialized basement membrane pivotal for skin integrity and function. However, increased inflammation in the DEJ is associated with the disruption and separation of this junction and sub-epidermal blistering. Granzyme B (GzmB) is a serine protease secreted by immune cells. Read More

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http://dx.doi.org/10.1038/s41598-018-28070-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6018769PMC
June 2018
23 Reads

Case Report of a 21-Year-Old Man With Epidermolysis Bullosa Acquisita.

J Cutan Med Surg 2018 May/Jun;22(3):356-358

2 Division of Dermatology and Department of Laboratory Medicine & Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada.

Epidermolysis bullosa acquisita (EBA) is a rare acquired type of mechanobullous disease affecting the dermal-epidermal junction (DEJ) of trauma prone acral surfaces. It manifests as tense vesicles, bullae, and milia and typically heals as atrophic hypo- or hyperpigmented scars. Classic noninflammatory mechanobullous EBA typically presents at a mean age of 48 years. Read More

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http://dx.doi.org/10.1177/1203475418756378DOI Listing
December 2018
2 Reads

An 81-Year-Old Woman with Recalcitrant Blisters.

Authors:
Daniel C Butler

Dermatopathology (Basel) 2018 Jan-Mar;5(1):1-5. Epub 2018 Jan 11.

Harvard Combined Dermatology Residency Program, and Departments of Pathology and Dermatology, Massachusetts General Hospital, Boston, MA, USA.

Epidermolysis bullosa acquisita (EBA) is a rare mucocutaneous blistering disorder with typical onset in adulthood. Diagnosis and management can be difficult owing to the variability in presentation and clinical manifestation. In this case, we explore a case of EBA as well as provide a general overview of the condition and its variants. Read More

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http://dx.doi.org/10.1159/000481529DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5920955PMC
January 2018
1 Read

Anti-Type VII Collagen Antibodies Are Identified in a Subpopulation of Bullous Pemphigoid Patients With Relapse.

Front Immunol 2018 21;9:570. Epub 2018 Mar 21.

Laboratory of Dermatology, Faculty of Medicine, University of Reims Champagne-Ardenne, Reims, France.

Bullous pemphigoid (BP) is an autoimmune bullous skin disease characterized by anti-BP180 and anti-BP230 autoantibodies (AAbs). Mucous membrane involvement is an uncommon clinical feature of BP which may evoke epidermolysis bullosa acquisita, another skin autoimmune disease characterized by anti-type VII collagen AAbs. We therefore evaluated the presence of anti-type VII collagen AAbs in the serum of BP patients with and without mucosal lesions at time of diagnosis and under therapy. Read More

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http://dx.doi.org/10.3389/fimmu.2018.00570DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5871753PMC
March 2018
10 Reads

Specific Inhibition of Complement Activation Significantly Ameliorates Autoimmune Blistering Disease in Mice.

Front Immunol 2018 16;9:535. Epub 2018 Mar 16.

Institute of Genetics, Department of Biology, University of Erlangen-Nuremberg, Erlangen, Germany.

Epidermolysis bullosa acquisita (EBA) is an antibody-mediated blistering skin disease associated with tissue-bound and circulating autoantibodies to type VII collagen (COL7). Transfer of antibodies against COL7 into mice results in a subepidermal blistering phenotype, strictly depending on the complement component C5. Further, activation predominantly by the alternative pathway is required to induce experimental EBA, as blistering was delayed and significantly ameliorated only in factor B mice. Read More

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http://dx.doi.org/10.3389/fimmu.2018.00535DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5865061PMC
March 2018
9 Reads

Discrepancies in the evaluation of incapacity for work in a patient with epidermolysis bullosa acquisita between public pension fund and occupational medicine expert raise the issue of competencies.

Authors:
Hrvoje Lalić

Arh Hig Rada Toksikol 2018 Mar;69(1):77-80

1Rijeka University Faculty of Medicine, Rijeka, Croatia.

Abstrat A 50-year-old female patient suffering from a severe form of epidermolysis bullosa acquisita (EBA) took legal action against the Croatian Pension Insurance Institute (CPII) in an attempt to overturn their assessment that she was no longer capable of working as a seamstress but still capable of doing administrative jobs. Her claim was that she was not capable of doing any job at all. She was first diagnosed EBA in 2000, and the disease progressed slowly with intermittent remissions. Read More

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http://dx.doi.org/10.2478/aiht-2018-69-3072DOI Listing
March 2018
2 Reads

The critical role of C5a as an initiator of neutrophil-mediated autoimmune inflammation of the joint and skin.

Semin Immunol 2018 06 27;37:21-29. Epub 2018 Mar 27.

Center for Immunology and Inflammatory Diseases, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

The deposition of IgG autoantibodies in peripheral tissues and the subsequent activation of the complement system, which leads to the accumulation of the anaphylatoxin C5a in these tissues, is a common hallmark of diverse autoimmune diseases, including rheumatoid arthritis (RA) and pemphigoid diseases (PDs). C5a is a potent chemoattractant for granulocytes and mice deficient in its precursor C5 or its receptor C5aR1 are resistant to granulocyte recruitment and, consequently, to tissue inflammation in several models of autoimmune diseases. However, the mechanism whereby C5a/C5aR regulates granulocyte recruitment in these diseases has remained elusive. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10445323173013
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http://dx.doi.org/10.1016/j.smim.2018.03.002DOI Listing
June 2018
4 Reads

Blistering diseases in the mature patient.

Clin Dermatol 2018 Mar - Apr;36(2):231-238. Epub 2017 Oct 3.

Department of Dermatovenereology, University Hospital Center Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia. Electronic address:

Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named "pemphigoid senilis"), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita, paraneoplastic pemphigus, pemphigus vulgaris, pemphigus foliaceus, linear IgA dermatosis, and dermatitis herpetiformis. Read More

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http://dx.doi.org/10.1016/j.clindermatol.2017.10.014DOI Listing
September 2018
11 Reads

Neutrophil Adhesion Is a Prerequisite for Antibody-Mediated Proteolytic Tissue Damage in Experimental Models of Epidermolysis Bullosa Acquisita.

J Invest Dermatol 2018 Sep 17;138(9):1990-1998. Epub 2018 Mar 17.

Priority Area Asthma and Allergy, Research Center Borstel, Borstel, Germany; Airway Research Center North, German Center for Lung Research (DZL). Electronic address:

Although uncontrolled proteolytic activity mediated by activated neutrophils is a major reason for tissue damage, therapeutic approaches using protease inhibitors are inefficient. Here, we investigated the role of the immune complex-induced neutrophil adhesion and protease release in tissue damage. We show both in vitro and in vivo that immune complex-mediated neutrophil adhesion to the target tissue depends on β integrins. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0022202X183172
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http://dx.doi.org/10.1016/j.jid.2018.03.1499DOI Listing
September 2018
14 Reads

The Autoimmune Skin Disease Bullous Pemphigoid: The Role of Mast Cells in Autoantibody-Induced Tissue Injury.

Front Immunol 2018 1;9:407. Epub 2018 Mar 1.

Department of Dermatology, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, United States.

Bullous pemphigoid (BP) is an autoimmune and inflammatory skin disease associated with subepidermal blistering and autoantibodies directed against the hemidesmosomal components BP180 and BP230. Animal models of BP were developed by passively transferring anti-BP180 IgG into mice, which recapitulates the key features of human BP. By using these model systems, key cellular and molecular events leading to the BP disease phenotype are identified, including binding of pathogenic IgG to its target, complement activation of the classical pathway, mast cell degranulation, and infiltration and activation of neutrophils. Read More

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http://dx.doi.org/10.3389/fimmu.2018.00407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5837973PMC
March 2018
9 Reads

Effectiveness and Safety of Rituximab in Recalcitrant Pemphigoid Diseases.

Front Immunol 2018 19;9:248. Epub 2018 Feb 19.

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

Introduction: Rituximab (RTX) is a monoclonal antibody targeting CD20, a transmembrane protein expressed on B cells, causing B cell depletion. RTX has shown great efficacy in studies of pemphigus vulgaris, but data of pemphigoid diseases are limited.

Objective: To assess the effectiveness and safety of RTX in pemphigoid diseases. Read More

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http://dx.doi.org/10.3389/fimmu.2018.00248DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5827539PMC
February 2018
6 Reads

Whole-Genome Expression Profiling in Skin Reveals SYK As a Key Regulator of Inflammation in Experimental Epidermolysis Bullosa Acquisita.

Front Immunol 2018 15;9:249. Epub 2018 Feb 15.

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

Because of the morbidity and limited therapeutic options of autoimmune diseases, there is a high, and thus far, unmet medical need for development of novel treatments. Pemphigoid diseases, such as epidermolysis bullosa acquisita (EBA), are prototypical autoimmune diseases that are caused by autoantibodies targeting structural proteins of the skin, leading to inflammation, mediated by myeloid cells. To identify novel treatment targets, we performed cutaneous genome-wide mRNA expression profiling in 190 outbred mice after EBA induction. Read More

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http://dx.doi.org/10.3389/fimmu.2018.00249DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5818881PMC
February 2018
19 Reads

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

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December 2017
11 Reads

Orf - a potential trigger for self-limiting epidermolysis bullosa acquisita-like blistering.

Br J Dermatol 2018 02;178(2):333

Department of Dermatology, Leicester Royal Infirmary, Leicester, LE1 5WW, U.K.

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http://dx.doi.org/10.1111/bjd.16055DOI Listing
February 2018
5 Reads

Treatment of Autoimmune Bullous Disorders in Pregnancy.

Am J Clin Dermatol 2018 Jun;19(3):391-403

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

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http://dx.doi.org/10.1007/s40257-018-0342-0DOI Listing
June 2018
7 Reads

Epidermal aspects of type VII collagen: Implications for dystrophic epidermolysis bullosa and epidermolysis bullosa acquisita.

J Dermatol 2018 May 20;45(5):515-521. 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

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http://dx.doi.org/10.1111/1346-8138.14222DOI Listing
May 2018
37 Reads

Response to 'Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita: a multicentre analysis': reply from authors.

Authors:
M Hertl T Hashimoto

Br J Dermatol 2018 02 10;178(2):573-574. Epub 2018 Jan 10.

Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan.

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http://dx.doi.org/10.1111/bjd.16169DOI Listing
February 2018
3 Reads

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

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http://dx.doi.org/10.1590/abd1806-4841.20175755DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5726665PMC
March 2018
10 Reads

Case of epidermolysis bullosa acquisita with concomitant anti-laminin-332 antibodies.

J Dermatol 2018 Apr 4;45(4):472-474. 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

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http://dx.doi.org/10.1111/1346-8138.14169DOI Listing
April 2018
10 Reads

Epidermolysis Bullosa (EB) Acquisita in an Adult Patient with Previously Unrecognized Mild Dystrophic EB and Biallelic COL7A1 Mutations.

Acta Derm Venereol 2018 Apr;98(4):411-415

Laboratory of Molecular and Cell Biology, Instituto Dermopatico dell'Immacolata-IRCCS, Via dei Monti di Creta, 104, IT-00167 Rome, Italy.

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 one 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

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http://dx.doi.org/10.2340/00015555-2851DOI Listing
April 2018
5 Reads

International Bullous Diseases Group: consensus on diagnostic criteria for epidermolysis bullosa acquisita.

Br J Dermatol 2018 Jul 8;179(1):30-41. Epub 2018 May 8.

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 the diagnosis of EBA essential.

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

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http://dx.doi.org/10.1111/bjd.16138DOI Listing
July 2018
3 Reads

Subepidermal autoimmune bullous diseases: overview, epidemiology, and associations.

Authors:
Khalaf Kridin

Immunol Res 2018 02;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

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http://dx.doi.org/10.1007/s12026-017-8975-2DOI Listing
February 2018
34 Reads

Serration pattern analysis for differentiating epidermolysis bullosa acquisita from other pemphigoid diseases.

J Am Acad Dermatol 2018 04 16;78(4):754-759.e6. 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

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http://dx.doi.org/10.1016/j.jaad.2017.11.029DOI Listing
April 2018
21 Reads

Efficacy of intravenous immunoglobulins for the treatment of mucous membrane pemphigoid-like epidermolysis bullosa acquisita.

Eur J Dermatol 2017 10;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.

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http://dx.doi.org/10.1684/ejd.2017.3118DOI Listing
October 2017
8 Reads

Response to 'Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita: a multicentre analysis'.

Br J Dermatol 2018 02 9;178(2):573. Epub 2018 Jan 9.

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

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http://dx.doi.org/10.1111/bjd.16023DOI Listing
February 2018
7 Reads

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

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http://dx.doi.org/10.1016/j.jid.2017.09.009DOI Listing
February 2018
14 Reads

Nanoparticles prepared from porcine cells support the healing of cutaneous inflammation in mice and wound re-epithelialization in human skin.

Exp Dermatol 2017 12 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

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http://dx.doi.org/10.1111/exd.13450DOI Listing
December 2017
19 Reads