Resolution in bullous pemphigoid

AbstractPemphigoid diseases are a group of autoimmune blistering skin diseases defined by an immune response against certain components of the dermal-epidermal adhesion complex. They are prototypical, autoantibody-driven, organ-specific diseases with the emergence of inflammatory skin lesions dependent on the recruitment of immune cells, particularly granulocytes, into the skin. During an acute flare of disease, inflammatory skin lesions typically progressing from erythema through urticarial plaques to subepidermal blisters erosions erupt and, finally, completely resolve, thus illustrating that resolution of inflammation is continuously executed in pemphigoid disease patients and can be directly monitored on the skin. Despite these superb conditions for examining resolution in pemphigoid diseases as paradigm diseases for antibody-induced tissue inflammation, the mechanisms of resolution in pemphigoid are underinvestigated and still largely elusive. In the last decade, mouse models for pemphigoid diseases were developed, which have been instrumental to identify several key pathways for the initiation of inflammation in these diseases. More recently, also protective pathways, specifically IL-10 and C5aR2 signalling on the molecular level and Tregs on the cellular level, counteracting skin inflammation have been highlighted and may contribute to the continuous execution of resolution in pemphigoid diseases. The upstream orchestrators of this process are currently under investiga...
Source: Seminars in Immunopathology - Category: Pathology Source Type: research

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AbstractDipeptidyl peptidase (DPP) ‐4 inhibitors, a class of oral hypoglycemic agents, are widely used, especially in Asian populations, as they have been shown to be well‐tolerated and to cause relatively few hypoglycemic events despite exerting a potent glucose‐lowering effect by promoting endogenous insulin secretion, beside s also having extra‐pancreatic effects. Meanwhile, use of DPP‐4 inhibitors has been reported to be associated with the development of bullous pemphigoid (BP), a rare autoimmune blistering skin disease1, even though the absolute increase in the risk of development of BP is relatively low.
Source: Journal of Diabetes Investigation - Category: Endocrinology Authors: Tags: Commentary Source Type: research
Bullous pemphigoid (BP) is the most prevalent autoimmune skin blistering disease and is characterized by the generation of autoantibodies against the hemidesmosomal proteins BP180 (type XVII collagen) and BP230. Most intriguingly, BP is distinct from other autoimmune diseases because it predominantly affects elderly individuals above the age of 75 years, raising the question why autoantibodies and the clinical lesions of BP emerges mostly in this later stage of life, even in individuals harboring known putative BP-associated germline gene variants. The mitochondrial genome (mtDNA) is a potential candidate to provide additi...
Source: Frontiers in Immunology - Category: Allergy & Immunology 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
Conclusions: One or more types of ADs developed in AE patients, and patients with anti-LGI1 encephalitis had a higher frequency of autoimmune comorbidities than those with anti-NMDAR encephalitis. And we found that autoimmune comorbidities did not affect the clinical course of AE.
Source: Frontiers in Neurology - Category: Neurology Source Type: research
In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, along with significant acral and cephalic distribution and mucosal involvement.
Source: Frontiers in Immunology - Category: Allergy & Immunology 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
Conclusions: This study describes the efficacy of immunosuppressants or biologics with an acceptable safety profile for OCP.
Source: Cornea - Category: Opthalmology Tags: Clinical Science Source Type: research
Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by autoantibodies targeting cellular adhesion molecules. While IgE autoantibodies are occasionally reported in other autoimmune blistering diseases, BP is unique in that most BP patients develop an IgE autoantibody response. It is not known why BP patients develop self-reactive IgE and the precise role of IgE in BP pathogenesis is not fully understood. However, clinical evidence suggests an association between elevated IgE antibodies and eosinophilia in BP patients. Since eosinophils are multipotent effector cells, capable cytotoxicity and immune mod...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Dermatologic immune-related adverse events (IRaes) are among the most frequent toxicities reported with anti –programmed cell death (PD)-1 or anti–programmed cell death ligand (PD-L1) therapies, affecting up to 40% of treated patients [1,2]. They have been associated with a variety of dermatologic manifestations including eczema-like rash, pruritus, lichenoid reactions, induction or reactivation of pre existing psoriasis, vitiligo and granulomatous reactions [3]. They may also induce or reactivate autoimmune skin diseases [4].
Source: European Journal of Cancer - Category: Cancer & Oncology Authors: Tags: Letter to the Editor Source Type: research
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