Publications by authors named "Anna Pira"

Background: Bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP) are rare autoimmune blistering disorders characterized by autoantibodies (autoAbs) targeting dermo-epidermal junction components such as BP180 and BP230. The differential diagnosis, based on both the time of appearance and the extension of cutaneous and/or mucosal lesions, is crucial to distinguish these diseases for improving therapy outcomes and delineating the correct prognosis; however, in some cases, it can be challenging. In addition, negative results obtained by commercially available enzyme-linked immunosorbent assays (ELISAs) with BP and MMP sera, especially from patients with ocular involvement, often delay diagnosis and treatment, leading to a greater risk of poor outcomes.

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Article Synopsis
  • Bullous pemphigoid (BP) is a prevalent autoimmune disease characterized by the presence of autoantibodies against hemidesmosomal components, specifically BP180 and BP230.
  • Despite studies indicating no direct link between COVID-19 vaccines and BP, there have been over 90 reported cases of vaccine-associated BP since the start of mass vaccinations.
  • An investigation involving 64 BP patients revealed a significant proportion developed the condition shortly after vaccination, suggesting that the vaccine might act as an accelerating factor for BP in genetically susceptible individuals.
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Bullous pemphigoid (BP) is the most common autoimmune bullous disease: it most commonly affects individuals over 70 years old and impacts severely on their quality of life. BP represents a paradigm for an organ-specific autoimmune disease and is characterized by circulating IgG autoantibodies to hemidesmosomal components: BP180 and BP230. While the crucial role of these autoantibodies in triggering BP inflammatory cascade is fully acknowledged, many ancillary etiological mechanisms need to be elucidated yet.

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Article Synopsis
  • Bullous pemphigoid (BP) is a rare autoimmune skin disorder, becoming more frequent with associations noted with certain diabetes medications called gliptins.
  • A study analyzed 30 idiopathic bullous pemphigoid (IBP) patients and 86 gliptin-associated BP (GABP) patients to explore genetic risk factors.
  • The research found a significant link between the HLA-DQB1*03:01 allele and both IBP and GABP, suggesting genetic markers that could indicate susceptibility to BP, particularly in individuals who have taken gliptins.
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Pemphigus is a life-threatening autoimmune blistering disease affecting skin and mucous membranes. Despite its etiopathogenesis remains largely unknown, several trigger and predisposing factors have been reported. Pemphigus is caused by autoantibodies that target desmoglein 1 and desmoglein 3, impacting desmosome function.

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Autoimmune bullous diseases (AIBDs) are a heterogeneous group of life-threatening disorders associated with subepidermal or intraepidermal blistering. Skin barrier alterations and prolonged immunosuppressive treatments increase the risk of infections in patients with AIBDs, who are considered fragile. COVID-19 pandemic had a heavy impact on these patients.

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Background: Bullous pemphigoid (BP) is the most common autoimmune-blistering disease, clinically characterized by erythematous urticarial plaques, blisters, and intense pruritus, induced by autoantibodies against two proteins of the dermo-epidermal junction, BP180 and BP230. A large number of autoimmune diseases are reported in the literature as BP comorbidities, such as multiple sclerosis, but only a few cases are in association with scleroderma and none in association with both.

Case Presentation: We present the case of a 68-year-old woman affected by multiple sclerosis and scleroderma who developed severe bullous pemphigoid with a bullous pemphigoid disease area index of 60 and high titers of anti-BP180 and anti-BP230 autoantibodies by enzyme-linked immunosorbent assays.

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