Updates on the Management of Autoimmune Blistering Diseases.

Updates on the Management of Autoimmune Blistering Diseases. Skin Therapy Lett. 2014 Oct;19(5):1-6 Authors: Hooten JN, Hall RP, Cardones AR Abstract Autoimmune blistering diseases are rare, but potentially debilitating cutaneous disorders characterized by varying degrees of mucosal and cutaneous bullae formation. Topical therapy is appropriate for mild and even some moderate disease activity, but systemic treatment can be considered for more extensive involvement. Corticosteroids remain the first-line systemic therapy for patients with moderate to severe bullous pemphigoid and pemphigus vulgaris. While the use of systemic steroids has dramatically reduced mortality from these two autoimmune blistering disorders, treatment is also associated with multiple side effects, especially when used long-term. Steroid sparing agents, therefore, are invaluable in inducing long-term remission while minimizing steroid associated side effects. Treatment must be tailored to the individual patient's condition, and several other factors must be carefully considered in choosing appropriate therapy: 1) diagnosis, 2) severity of the condition and body site affected, 3) presence of comorbidities, and 4) ability to tolerate systemic therapy. PMID: 25405675 [PubMed - as supplied by publisher]
Source: Skin Therapy Letter - Category: Dermatology Authors: Tags: Skin Therapy Lett Source Type: research

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Abstract Numerous epidemiological studies have suggested a link between vitamin D deficiency and the development of various autoimmune diseases, including diabetes mellitus type 1, rheumatoid arthritis, inflammatory bowel disease, multiple sclerosis or systemic lupus erythematosus. More recently, such a link has been also proposed for autoimmune bullous diseases (AIBD). This is a relatively rare and potentially life-threatening, organ-specific group of inflammatory skin diseases characterized by the presence of tissue-bound and circulating autoantibodies against various molecules present in desmosomes (in pemphigu...
Source: Acta Biochim Pol - Category: Biochemistry Authors: Tags: Acta Biochim Pol Source Type: research
To the Editor: Pemphigus is an autoimmune blistering disease with substantial pain, which affects quality of life.1-3 Previous reports and anecdotal clinical observations suggest that patients with  pemphigus with cutaneous involvement frequently experience pruritus. We sought to elucidate the prevalence and predictors of itch in pemphigus compared with bullous pemphigoid (BP), for which pruritus has been well documented.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Tags: Letter Source Type: research
dwig 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: 31...
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
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
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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
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