Keratinocyte footprint assay discriminates anti-laminin-332 pemphigoid from all other forms of pemphigoid diseases.

CONCLUSIONS: The keratinocyte footprint assay is a fast and specific assay to confirm or rule out the presence of anti-laminin-332 autoantibodies. This article is protected by copyright. All rights reserved. PMID: 31090065 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research

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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 are usually administered at a dose of 2g per kg body weight distributed over two to five 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 ...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Publication date: Available online 7 June 2019Source: Autoimmunity ReviewsAuthor(s): Khalaf Kridin, Diana Kneiber, Eric H. Kowalski, Manuel Valdebran, Kyle T. AmberAbstractEpidermolysis 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. Patients with mechanobullous EBA develop non-inflammatory bullae and erosions at sites of trauma while patients w...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
Abstract: 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. A flowchart, based on the experience and joint discussion among the authors of this consensus, was constructed to provide treatment...
Source: Anais Brasileiros de Dermatologia - Category: Dermatology Source Type: research
The presence of one or several autoantigen(s) and a response by the adaptive immune system are the key criteria to classify a pathology as an autoimmune disease. The list of entities fulfilling this criterion is currently growing in the light of recent advancements in the pathogenetic understanding of a number of important dermatoses. The role of autoreactive T-lymphocytes differs amongst these pathologies. While they are directly involved as effector cells attacking and sometimes killing their respective target in some diseases (e.g. vitiligo), they provide help to B-lymphocytes, which in turn produce the pathogenic autor...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Bullous pemphigoid (BP) is a common autoimmune blistering disease in which autoantibodies target the hemidesmosomal components BP180 and/or BP230 in basal keratinocytes. In BP, 80 to 90% of autoantibodies target the juxtamembranous extracellular non-collagenous 16th A (NC16A) domain of BP180. Recently, the administration of dipeptidyl peptidase-IV inhibitors (DPP4i), which are widely used as antihyperglycemic drugs, has been recognized to be a causative factor for BP. DPP4i-associated BP (DPP4i-BP) autoantibodies tend to target epitopes on non-NC16A regions of BP180, and the pathomechanism for the development of the unique...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Authors: Feizi S, Roshandel D Abstract 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. Ocular pemphigus vulgaris exhibits intraepithelial activity, whereas the autoimmune activity in linear immunoglobulin A disease, m...
Source: Journal of Ophthalmic and Vision Research - Category: Opthalmology Tags: J Ophthalmic Vis Res Source Type: research
This article is protected by copyright. All rights reserved. PMID: 31124130 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
We report a 68-year-old Thai woman diagnosed and treated for bullous pemphigoid (BP) for 11 months who recently presented with a 3-day history of extensive hemorrhagic bullae and large intra-oral buccal hematoma. Laboratory investigations confirmed a prolonged activated partial thromboplastin time, a low factor VIII level, a high factor VIII inhibitor level, and elevated anti-BPAG180 and anti-BPAG230 titers, confirming the diagnosis of BP associated with AHA. Immunosuppressive therapy with systemic corticosteroids and cyclophosphamide combined with bypassing agents for bleeding control resulted in significant clinical impr...
Source: Case Reports in Dermatology - Category: Dermatology Source Type: research
CONCLUSION: When prescribing PD-1 inhibitors, physicians should monitor closely for symptoms of bullous pemphigoid. PMID: 31088310 [PubMed - as supplied by publisher]
Source: Expert Opinion on Drug Safety - Category: Drugs & Pharmacology Tags: Expert Opin Drug Saf Source Type: research
Abstract: Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities a...
Source: Anais Brasileiros de Dermatologia - Category: Dermatology Source Type: research
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