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Pemphigoid

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1. A randomised controlled trial to compare the safety, effectiveness and cost effectiveness of doxycycline (200 mg/day) with oral prednisolone (0.5 mg/kg/day) for initial treatment of bullous pemphigoid: the Bullous Pemphigoid Steroids and Tetracyclines (BL

A randomised controlled trial to compare the safety, effectiveness and cost effectiveness of doxycycline (200 mg/day) with oral prednisolone (0.5 mg/kg/day) for initial treatment of bullous pemphigoid: the Bullous Pemphigoid Steroids and Tetracyclines (BL A randomised controlled trial to compare the safety, effectiveness and cost effectiveness of doxycycline (200 mg/day) with oral prednisolone (0.5 mg/kg/day) for initial treatment of bullous pemphigoid: the Bullous Pemphigoid Steroids

2017 NIHR HTA programme

2. Characteristics of patients with bullous pemphigoid: comparison of classic bullous pemphigoid to non-bullous pemphigoid. (PubMed)

Characteristics of patients with bullous pemphigoid: comparison of classic bullous pemphigoid to non-bullous pemphigoid. Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease. Patients occasionally present with a clinical picture of pruritus/urticaria alone for months and do not even develop blisters over time. Only few studies have investigated this subgroup of non-bullous pemphigoid (NBP).To evaluate the demographic and clinical characteristics of BP patients

2019 Journal of the European Academy of Dermatology and Venereology

5. Bullous pemphigoid

Bullous pemphigoid Bullous pemphigoid - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Bullous pemphigoid Last reviewed: February 2019 Last updated: November 2018 Summary Typically occurs in older people and has a distinctive clinical appearance. There are several clinical variants, 1 of which occurs in childhood. In the prodromal, non-bullous phase, pruritus of variable intensity may be accompanied by eczematous (...) of existing blisters and erosions, and to control the associated pruritus. Topical and systemic corticosteroids, as well as other immunosuppressive and anti-inflammatory agents, are used. Definition Bullous pemphigoid is a chronic, acquired autoimmune blistering disease characterised by auto-antibodies against hemidesmosomal antigens, resulting in the formation of a sub-epidermal blister. Liu Z. Are anti-BP180 IgG1 or IgG4 autoantibodies pathogenic? J Invest Dermatol. 2002;119:989-990. History and exam

2018 BMJ Best Practice

6. Bullous pemphigoid

Bullous pemphigoid Bullous pemphigoid - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Bullous pemphigoid Last reviewed: February 2019 Last updated: November 2018 Summary Typically occurs in older people and has a distinctive clinical appearance. There are several clinical variants, 1 of which occurs in childhood. In the prodromal, non-bullous phase, pruritus of variable intensity may be accompanied by eczematous (...) of existing blisters and erosions, and to control the associated pruritus. Topical and systemic corticosteroids, as well as other immunosuppressive and anti-inflammatory agents, are used. Definition Bullous pemphigoid is a chronic, acquired autoimmune blistering disease characterised by auto-antibodies against hemidesmosomal antigens, resulting in the formation of a sub-epidermal blister. Liu Z. Are anti-BP180 IgG1 or IgG4 autoantibodies pathogenic? J Invest Dermatol. 2002;119:989-990. History and exam

2018 BMJ Best Practice

7. Mucous Membrane Pemphigoid, Bullous Pemphigoid, and Anti-programmed Death-1/ Programmed Death-Ligand 1: A Case Report of an Elderly Woman With Mucous Membrane Pemphigoid Developing After Pembrolizumab Therapy for Metastatic Melanoma and Review of the Lite (PubMed)

Mucous Membrane Pemphigoid, Bullous Pemphigoid, and Anti-programmed Death-1/ Programmed Death-Ligand 1: A Case Report of an Elderly Woman With Mucous Membrane Pemphigoid Developing After Pembrolizumab Therapy for Metastatic Melanoma and Review of the Lite An 83-year-old patient developed erosions and a blister of the gingival mucous membrane, 6 months after discontinuation of the anti-programmed death-1 (anti PD-1) pembrolizumab therapy administered for 10 months for a metastatic melanoma (...) . A diagnosis of mild mucous membrane pemphigoid (MMP) was made. Complete remission of MMP was rapidly obtained with minimal therapy (doxycycline). MMP remained in complete remission after a 3-month follow-up since discontinuation of the doxycycline therapy and no evidence of relapse of the melanoma was observed after a 14-month follow-up since discontinuation of the pembrolizumab therapy. The widespread use of anti PD-1 and anti-programmed death-ligand-1 (PD-L1) in several malignancies reveals new adverse

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2018 Frontiers in medicine

8. Gliptin-associated Bullous Pemphigoid and the Expression of Dipeptidyl Peptidase-4/CD26 in Bullous Pemphigoid. (PubMed)

Gliptin-associated Bullous Pemphigoid and the Expression of Dipeptidyl Peptidase-4/CD26 in Bullous Pemphigoid. Dipeptidyl peptidase-4 inhibitors (DPP-4i or gliptins) increase the risk of developing bullous pemphigoid (BP). To clarify, whether gliptin-associated BP has special features, we analyzed the clinical, histopathological and immunological features of 27 BP patients, 10 of which previously used gliptin medication. Compared to those who had not previously received gliptins, subjects who

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2019 Acta Dermato-Venereologica

9. Circulating Bullous Pemphigoid Autoantibodies in the Setting of Negative Direct Immunofluorescence Findings for Bullous Pemphigoid: A Single-Center Retrospective Review. (PubMed)

Circulating Bullous Pemphigoid Autoantibodies in the Setting of Negative Direct Immunofluorescence Findings for Bullous Pemphigoid: A Single-Center Retrospective Review. Bullous pemphigoid (BP) autoantibody levels are generally elevated in patients with BP but can be present nonspecifically in patients without BP.To analyze elevated levels of BP180 or BP230 autoantibodies and clinical findings for patients who had negative direct immunofluorescence (DIF) studies.We retrospectively reviewed

2019 Journal of American Academy of Dermatology

10. Keratinocyte footprint assay discriminates anti-laminin-332 pemphigoid from all other forms of pemphigoid diseases. (PubMed)

Keratinocyte footprint assay discriminates anti-laminin-332 pemphigoid from all other forms of pemphigoid diseases. Anti-laminin-332 mucous membrane pemphigoid is a chronic severe pemphigoid disease characterized by autoantibodies to laminin-332. At present no commercial assay is available to demonstrate anti-laminin-332 antibodies and diagnosis relies on in-house techniques with limited sensitivities.In order to move, keratinocytes cultured in vitro secrete laminin-332 to attach to the culture (...) -laminin-332 autoantibodies it was converted into a daily available test based on binding of IgG to dried coverslips that can be stored frozen. The staining patterns of sera from anti-laminin-332 pemphigoid patients were then compared to those of sera of patients with other autoimmune bullous diseases and normal human sera.IgG of all anti-laminin-332 pemphigoid sera (n=16) bound to laminin-332 footprints while all normal human controls (n=55) were negative. From the sera of patients with other diseases

2019 British Journal of Dermatology

11. Increased frequency of multiple sclerosis among bullous pemphigoid patients. A population-based cohort study on comorbidities anchored around the diagnosis of bullous pemphigoid. (PubMed)

Increased frequency of multiple sclerosis among bullous pemphigoid patients. A population-based cohort study on comorbidities anchored around the diagnosis of bullous pemphigoid. Bullous pemphigoid (BP) is a disease of the elderly and may be associated with neurological and cardiovascular diseases and diabetes. Mortality rates strongly exceed those of the background population.To investigate the frequency of comorbidities and their temporal relation to BP.A register-based matched-cohort study

2017 British Journal of Dermatology

12. Neurodegenerative disorders, bullous pemphigoid and psoriasis: a comparative study in ethnic Poles indicates that Parkinson’s disease is more relevant to bullous pemphigoid (PubMed)

Neurodegenerative disorders, bullous pemphigoid and psoriasis: a comparative study in ethnic Poles indicates that Parkinson’s disease is more relevant to bullous pemphigoid Bullous pemphigoid (BP) is an autoimmune blistering dermatosis of the elderly with autoimmunity to hemidesmosomal proteins, BP180 and BP230, which are expressed also in neuronal tissue.The aim here was to retrospectively compare the prevalence of neurodegenerative disorders (ND), particularly Parkinson's disease (PD

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2017 Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii

13. A randomised controlled trial to compare the safety, effectiveness and cost-effectiveness of doxycycline (200 mg/day) with that of oral prednisolone (0.5 mg/kg/day) for initial treatment of bullous pemphigoid: the Bullous Pemphigoid Steroids and Tetra (PubMed)

A randomised controlled trial to compare the safety, effectiveness and cost-effectiveness of doxycycline (200 mg/day) with that of oral prednisolone (0.5 mg/kg/day) for initial treatment of bullous pemphigoid: the Bullous Pemphigoid Steroids and Tetra Bullous pemphigoid (BP) is an autoimmune blistering skin disorder with increased morbidity and mortality in the elderly.To evaluate the effectiveness, safety and cost-effectiveness of a strategy of initiating BP treatment with oral doxycycline

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2017 Health technology assessment (Winchester, England)

14. Pemphigoid gestationis and intravenous immunoglobulin therapy (PubMed)

Pemphigoid gestationis and intravenous immunoglobulin therapy Pemphigoid gestationis, which is also known as herpes gestationis, is a rare, pregnancy-associated, autoimmune bullous disease. Treatment depends on the severity of the disease for each patient and the safety and use of these drugs during pregnancy and breastfeeding must be taken into consideration to guide their use. We describe the therapeutic response of two cases of pemphigoid gestationis that did not respond to conventional (...) immunosuppressive therapy or adverse effects limited their use. Both patients eventually received treatment with intravenous immunoglobulin therapy, which resulted in clinical remission. This clinical improvement with disappearance of lesions and a reduction in pruritus was paralleled in a decline in Bullous Pemphigoid Disease Activity Index activity scores, which is a validated scoring system to measure the related condition, bullous pemphigoid.

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2018 International journal of women's dermatology

15. Disseminated Nocardiosis with retinal abscess in a patient treated for bullous pemphigoid (PubMed)

Disseminated Nocardiosis with retinal abscess in a patient treated for bullous pemphigoid To report a case of disseminated Nocardiosis with retinal and intracranial lesions.A 49-year-old woman immunosuppressed because of treatment given for bullous pemphigoid presented with altered mental status and multiple intracranial lesions on imaging. The patient was found to have multiple retinal lesions in both eyes, including a subretinal abscess in the right eye. The patient underwent brain biopsy

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2018 American journal of ophthalmology case reports

16. Adrenocorticotropic hormone analogue as novel treatment regimen in ocular cicatricial pemphigoid (PubMed)

Adrenocorticotropic hormone analogue as novel treatment regimen in ocular cicatricial pemphigoid To report the clinical outcome of a patient with ocular cicatricial pemphigoid, treated with adrenocorticotropic hormone gel.A 75-year-old female with a biopsy proven ocular cicatricial pemphigoid (OCP) presented with bilateral conjunctival inflammation, fornix shortening, subepithelial fibrosis and corneal scarring. The patient was previously treated with topical steroids, topical cyclosporine

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2018 American journal of ophthalmology case reports

17. Vulvar lichen planus pemphigoides (PubMed)

Vulvar lichen planus pemphigoides Lichen planus pemphigoides (LPP) is a rare blistering disease with features of both lichen planus and bullous pemphigoid. LPP typically appears on the extremities and occasionally involves the oral mucosa. Herein, we describe a case of LPP of the vulva of an 80-year-old woman, an uncommon location for this disease process. This clinical scenario can be confused with a number of similarly appearing entities such as erosive vulvar lichen planus, mucous membrane (...) pemphigoid, and erosive lichen sclerosus et atrophicus. In fact, our patient carried a diagnosis of lichen sclerosus by an outside physician for 2 years prior to being properly diagnosed and treated. A detailed discussion of the epidemiology, clinical, and pathogenesis as well as the histologic and immunofluorescence characteristics of this uncommon diagnosis is presented. Our case emphasizes the necessity of microscopic analysis to differentiate lookalike disease states when making a diagnosis

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2017 International journal of women's dermatology

18. The use of biological agents in the treatment of oral lesions due to pemphigus, mucous membrane pemphigoid (MMP), oral lichen planus (OLP), recurrent aphthous ulcers and Beh‡et's disease: a systematic review

The use of biological agents in the treatment of oral lesions due to pemphigus, mucous membrane pemphigoid (MMP), oral lichen planus (OLP), recurrent aphthous ulcers and Beh‡et's disease: a systematic review Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears

2019 PROSPERO

19. Increased risk of venous thromboembolism in patients with bullous pemphigoid. The INVENTEP (INcidence of VENous ThromboEmbolism in bullous Pemphigoid) study. (PubMed)

Increased risk of venous thromboembolism in patients with bullous pemphigoid. The INVENTEP (INcidence of VENous ThromboEmbolism in bullous Pemphigoid) study. Activation of blood coagulation has been demonstrated in bullous pemphigoid (BP), a rare autoimmune blistering disease, potentially leading to a prothrombotic state. In order to evaluate the incidence of venous thromboembolism (VTE) in BP, a cohort study was carried out on 432 BP patients (59% females; median age 76 years, interquartile

2016 Thrombosis and haemostasis

20. Biochip detection of BP180 autoantibodies in blister fluid for the serodiagnosis of bullous pemphigoid: A pilot study. (PubMed)

Biochip detection of BP180 autoantibodies in blister fluid for the serodiagnosis of bullous pemphigoid: A pilot study. Bullous pemphigoid is an autoimmune subepidermal blistering skin disease immunologically defined by autoantibodies directed against basement membrane zone antigens, the main of which is BP180. Laboratory tests are essential for diagnosis and include direct immunofluorescence and serologic assessments with indirect immunofluorescence and ELISA. Serology may be performed (...) on blister fluid, in alternative to blood serum. This study investigated the use of a Biochip-based indirect immunofluorescence approach for the serum diagnosis of bullous pemphigoid on blister fluid. We compared the results using the Biochip-method with the ELISA detection of bullous pemphigoid-180 autoantibodies in blister fluid and observed a perfect correlation between these 2 methods in our group of 13 patients with clinical and direct immunofluorescence diagnosis of bullous pemphigoid. The Biochip

2019 Medicine

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