Assessment of the healthcare costs for pemphigus and bullous pemphigoid patients in an academic center in Germany.

Assessment of the healthcare costs for pemphigus and bullous pemphigoid patients in an academic center in Germany. Br J Dermatol. 2019 Nov 20;: Authors: Ständer S, Färber B, Radeke S, Schmidt E, Zillikens D, Ludwig RJ Abstract The autoimmune skin blistering diseases pemphigus (vulgaris and foliaceus) and bullous pemphigoid (BP) pose a high burden on affected patients. With current treatment options, induction of remission is achieved in most patients. However, prolonged immunosuppression is required to maintain remission, and treatment-related morbidity and mortality further adds to the patients' burden1,2 . Hence, development of novel therapeutic strategies that are effective and safe is highly warranted. Recently, insights into pemphigus and BP pathogenesis identified new therapeutic targets and drugs3 . PMID: 31749141 [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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Autoimmune blistering diseases (AIBDs) of the skin are characterized by autoantibodies against different intra-/extracellular structures within the epidermis and at the basement membrane zone (BMZ). Binding of the antibodies to their target antigen leads to inflammation at the respective binding site and degradation of these structures, resulting in the separation of the affected skin layers. Clinically, blistering, erythema and lesions of the skin and/or mucous membranes can be observed. Based on the localization of the autoantigen, AIBDs can be divided into pemphigus (intra-epidermal blistering diseases) and pemphigoid d...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
This study aimed to evaluate the association of autoimmune bullous diseases [bullous pemphigoid (BP) and pemphigus vulgaris (PV)] with radiotherapy (RT) among patients with breast cancer from a population-based Taiwanese database. The case –control study included 365 women with BP or PV and 1460 randomly selected propensity score-matched controls without BP or PV. We compared the prevalences of prior RT and breast cancer between the cases and controls. In addition, we performed multivariable logistic regression analysis to calculate the odds ratios (ORs) and 95% confidence intervals (CIs) for developing BP or PV acco...
Source: Archives of Dermatological Research - Category: Dermatology Source Type: research
Bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP) are rare chronic autoimmune disorders characterized by subepidermal blistering. For the United States, there is a limited amount of studies in BP and MMP that address disease demographics and clinical data. In order to more comprehensively examine disease demographics and clinical factors, we performed a retrospective analysis of patient-reported data of 138 BP and 165 MMP patients enrolled in the International Pemphigus &Pemphigoid Foundation (IPPF) disease registry from 2010–2016. Patient-reported data was compared to Physician/Investigator reported d...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Conclusions: Rituximab is both safe and effective for the treatment of acquired angioedema with C1-inhibitor deficiency, ANCA-associated vasculitis, autoimmune hemolytic anemia, Behçet’s disease, bullous pemphigoid, Castleman’s disease, cryoglobulinemia, Goodpasture’s disease, IgG4-related disease, immune thrombocytopenia, juvenile idiopathic arthritis, membraneous nephropathy, relapsing-remitting multiple sclerosis, myasthenia gravis, nephrotic syndrome, neuromyelitis optica, pemphigus, rheumatoid arthritis, spondyloarthropathy, and systemic sclerosis. Conversely, rituximab failed to show an effec...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Autoimmune bullous dermatoses (AIBD) comprise a spectrum of blistering diseases, the most common being pemphigus and pemphigoid. Their autoimmune nature has been well documented but the etiological role of genetic background orvarious environmental factors remains unclear. The occurrence of infections in AIBD patients is probable regarding their immunosuppressive treatment but whether some viruses have the potential to induce pemphigus orpemphigoid is questionable. The aim of our study was to identify the presence of antiviral serum antibodies in newly diagnosed pemphigus and pemphigoid patients prior to immunosuppressive therapy.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Adaptive Immunity and Autoimmunity Source Type: research
Autoimmune bullous dermatoses (AIBD) include a series of typical organ-specific autoimmune diseases characterized by extensive mucocutaneous blisters. It is generally accepted to be caused by pathological autoantibodies that directly target specific adhesion components of the skin or the adjacent mucous membranes. Both innate and adaptive immune systems are critically involved in the misguided immune response against self-antigens. Recent studies have indicated that the dysfunction of regulatory T cells, regulatory B cells, and complement regulatory proteins that play essential roles in maintaining a healthy immune environ...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
AbstractAutoimmune bullous diseases are a group of chronic inflammatory disorders caused by autoantibodies targeted against structural proteins of the desmosomal and hemidesmosomal plaques in the skin and mucosa, leading to intra-epithelial or subepithelial blistering. The oral mucosa is frequently affected in these diseases, in particular, in mucous membrane pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus. The clinical symptoms are heterogeneous and may present with erythema, blisters, erosions, and ulcers localized anywhere on the oral mucosa, and lead to severe complaints for the patients including pain, dy...
Source: American Journal of Clinical Dermatology - Category: Dermatology 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
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
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
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