Epidermolysis bullosa acquisita and anti-p200 pemphigoid as major subepidermal autoimmune bullous diseases diagnosed by floor binding on indirect immunofluorescence microscopy using human salt-split skin

Conclusion: In this study, we report three cases of anti-p200 pemphigoid from India. These cases, though indistinguishable clinically from bullous pemphigoid, revealed a floor-binding pattern on indirect immunofluorescence using salt-split skin.
Source: Indian Journal of Dermatology, Venereology and Leprology - Category: Dermatology Authors: Source Type: research

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ConclusionsThe clinico-epidemiological characteristics vary among the types of autoimmune bullous diseases. Females were more predominant in most of them. Sudanese patients tended in general to present at a younger age than other populations. The pool of Sudanese patients with autoimmune bullous diseases is large which requires investigation for the local risk factors and presents a field for future trials.
Source: PLoS One - Category: Biomedical Science Authors: Source Type: research
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...
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Original Article Source Type: research
Subepidermal 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 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.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Source Type: research
In conclusion, serrated pattern analysis using direct immunofluorescence has a high detection frequency and specificity for epidermolysis bullosa acquisita and will further facilitate the diagnosis of latter disorder.PMID:33491096 | DOI:10.2340/00015555-3748
Source: Acta Derm Venereol A... - Category: Dermatology Authors: Source Type: research
ik CD Abstract 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 prototypic...
Source: Journal of Autoimmunity - Category: Allergy & Immunology Authors: Tags: J Autoimmun Source Type: research
Autoimmune blistering diseases (AIBDs), mainly including epidermolysis bullosa acquisita (EBA), pemphigus Vulgaris (PV) and bullous pemphigoid (BP), are a large class of autoimmune diseases presenting the blistering eruptions on the skin and mucosa membrane. Circulating autoantibodies play a critical role in the pathogenesis, antibody-specific B cells and CD4+T cells involved as well. While vitiligo, a common pigmentation disorder, is mostly considered as CD8+T cell-mediated, with multiple melanocyte-derived autoantibodies also detectable in part of the patients, although its pathogenicity remains undetermined.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Adaptive and Auto-Immunity Source Type: research
Autoimmune blistering diseases (AIBDs) are a group of rare acquired blistering skin diseases, which are divided into five major subtypes based on the clinical appearance and pathology: pemphigus diseases, bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), dermatitis herpetiformis (DH) and Linear IgA bullous dermatosis (LigA). Current understanding has been greatly increased by genetic investigations mainly focus on the HLA in various populations. We have conducted the HLA association studies on different subtypes of AIBDs in Chinese population by using Next-generation (NGS) based HLA typing methods.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Patient Population Research Source Type: research
Abstract Collagen XVII (COL17) is a hemidesmosomal transmembrane protein in the skin, which, in several autoimmune blistering skin diseases, may be targeted by autoantibodies. In addition, loss-of-function mutations in the COL17A1 gene induce a subtype of junctional epidermolysis bullosa. The extracellular domain of COL17 can be physiologically cleaved from the cell surface by ADAM family proteins in a process known as ectodomain shedding. COL17 ectodomain shedding is thought to be associated with the migration and proliferation of keratinocytes. Furthermore, the C-terminal cleavage of COL17 may be associated with...
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research
The epidermis is a stratified epithelium whose homeostasis is coordinated by stem cell self-renewal, cell proliferation and cell differentiation. The epidermis is attached to its covering dermis by basement membrane zone proteins. The inherited dysfunction of those proteins (as seen in epidermolysis bullosa) or autoimmune reactions to those proteins (as seen in pemphigoid diseases) typically results in skin in which even gentle mechanical forces produce blisters at the basement membrane zone. However, the healing processes of subepidermal blisters have not been fully characterized.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Stem Cells, Skin Appendages and Tissue Regeneration Source Type: research
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. 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. Deposition of complement components is routinely used as a diagnostic marker for AIBD. Knowledge from different animal models mimicking AIBD and depositio...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
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