S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid

Eming, Ruediger; Sticherling, Michael; Hofmann, Silke; Hunzelmann, Nicolas; Kern, Johannes S.; Kramer, Harald; Pfeiffer, Christiane; Schuster, Volker; Zillikens, Detlef; Goebeler, Matthias; Hertl, Michael; Nast, Alexander; Orzechowski, Hans‐Dieter; Sárdy, Miklós; Schmidt, Enno; Sitaru, Cassian; Sporbeck, Birte; Worm, Margitta

doi:10.1111/ddg.12606

Cited by 77 publications

(103 citation statements)

References 26 publications

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“…The gold standard for the diagnosis is DIF from perilesional skin, demonstrating a linear deposition of IgG along the BMZ. Also IgG autoantibodies targeting the two main autoantigens can be detected in the patients' sera via ELISA . Thus, previously IgG autoantibodies have been considered to be the major players in the pathogenesis of BP.…”

Section: Discussion and Review Of Literaturementioning

confidence: 99%

“…There are numerous therapeutic options available. Several national and international guidelines have been already published, and, depending on comorbidities of the patients, effectively guide the choice of therapy . The standard therapies consist of topical and systemic corticosteroids, and other immunosuppressive agents like azathioprine, mycophenolate mofetile, dapsone, methotrexate, anti‐inflammatory antibiotics (e.g.…”

Section: Introductionmentioning

confidence: 99%

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Successful treatment of bullous pemphigoid with omalizumab as corticosteroid‐sparing agent: report of two cases and review of literature

Balakirski

Alkhateeb

Merk

et al. 2016

Acad Dermatol Venereol

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Until recently IgG autoantibodies against the basal membrane proteins BP180 und BP230 were considered to be causative in the pathogenesis of BP. However, new in vitro studies as well as data from experimental mouse models have indicated that in addition to specific IgG, also IgE antibodies against BP180 and BP230 play a role in the development of this disease. Based on these new findings, new treatment modalities of BP became possible.

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Section: Discussion and Review Of Literaturementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Successful treatment of bullous pemphigoid with omalizumab as corticosteroid‐sparing agent: report of two cases and review of literature

Balakirski

Alkhateeb

Merk

et al. 2016

Acad Dermatol Venereol

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“…[31,32] Treatment options are mainly oral and/or topical superpotent corticosteroids frequently combined with immunomodulants such as dapsone and tetracyclines or immunosuppressant agents like azathioprine and methotrexate. [33,34] Recent reports about the successful treatment of individual BP patients with the anti-IgE antibody omalizumab [35][36][37][38][39][40] suggest a pathogenic relevance of IgE autoantibodies.…”

Section: Introductionmentioning

confidence: 99%

IgE-mediated mechanisms in bullous pemphigoid and other autoimmune bullous diseases

Beek

Schulze

Zillikens

et al. 2015

Expert Review of Clinical Immunology

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Autoimmune bullous diseases (AIBDs) are characterized by autoantibodies against structural proteins of the dermal-epidermal junction (in pemphigoid diseases) and the epidermal/ epithelial desmosomes (in pemphigus diseases). By far, the most common AIBD is bullous pemphigoid, which is immunopathologically characterized by autoantibodies against BP180 (type XVII collagen) and BP230. IgG and, to a lesser extent, IgA autoantibodies are the major autoantibody isotypes in these disorders. IgE autoantibodies are increasingly reported in particular in bullous pemphigoid. The development of specific and sensitive anti-BP180 IgE ELISA systems, the report of two experimental murine models employing IgE autoantibodies against BP180, and the successful treatment of bullous pemphigoid with the anti-IgE antibody omalizumab have raised interest in the role of IgE autoantibodies and the modulation of their production in AIBDs. Here, the relevance of IgE autoantibodies in the diagnosis, pathophysiology, and treatment decisions of AIBDs, with a focus on bullous pemphigoid, is reviewed. ARTICLE HISTORY

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“…Hastalığın primer tedavisi yüksek potent topikal kortikosteroidler olsa da genel olarak hastalıktan etkilenen ileri yaş popülasyonu için topikal tedavi kullanımı pratik değildir ve özellikle yaygın hastalığı olan olgularda kısa süreli sistemik steroid tedavisi öncelikli olarak tercih edilmektedir. Remisyon ve relapslarla karakterize hastalığın kronik seyri nedeniyle steroid tedavisinin potansiyel yan etkilerinden korunmak için ek immünsüpresif (mikofenolat mofetil, azathioprine, metotreksat) ve anti-enflamatuvar (tetrasiklin, nikotinamid, dapson) ajanlar ve konvansiyonel tedavilere yanıtsız olgularda intravenöz immünoglobulin (IVIG) tedavisi kullanılabilmektedir [7][8][9] . BP tanılı olguların klinik ve demografik özelliklerini irdeleyen çalışmalar hastalık etiyopatogenezinin anlaşılmasında ve yönetiminde, optimal tedavinin belirlenmesinde önem arz etmektedir.…”

Section: Introductionunclassified

Retrospective analysis of the forty-six patients with bullous pemphigoid followed-up in our clinic

Yazıcı¹,

Başkan²,

Tunali³

et al. 2016

TURKDERM

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Background and Design: Bullous pemphigoid (BP) is an autoimmune disease characterised by widespread itchy plaques and subepithelial blisterings and usually affects the elderly population. Due to the chronic nature of the disease, to prevent the side effects of chronic steroid treatment, adjuvant immunosuppressive (mycophenolate mofetil, azathioprine, methotrexate) and anti-inflammatory (tetracycline, nicotinamide, dapsone) agents may be used. In this study, we retrospectively evaluated the clinical and demographic characteristics and applied treatments of 46 patients with the diagnosis of BP and compared with literature data. Materials and Methods: We retrospectively evaluated the records of 46 patients who received clinical and histopathological diagnosis of BP and followed up in our clinic between 2006 and 2013. Results: Of the 46 patients, 22 were female and 24 male. The mean age of onset was 69.54 years (range: 18-105). The duration of the lesion ranged from 1 week to 10 months with a median duration of 8 weeks. The most frequent comorbid chronic disease was hypertension detected in 28 (60.8%) patients. Only four patients had a history of malignancy before the onset of the disease. Nineteen patients (42%) used more than 5 agents for comorbid diseases. Thirty-two patients (69.5%) used systemic corticosteroids alone and ten (22%) patients needed additional adjuvant therapies. Conclusion: BP is a major cause of morbidity in the elderly population receiving multiple drug treatment. To avoid the side effects of steroid therapy, especially in patients with severe disease, short-term use of additional immunosuppressive agents appears to be safe and effective.

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S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid

Cited by 77 publications

References 26 publications

Successful treatment of bullous pemphigoid with omalizumab as corticosteroid‐sparing agent: report of two cases and review of literature

Successful treatment of bullous pemphigoid with omalizumab as corticosteroid‐sparing agent: report of two cases and review of literature

IgE-mediated mechanisms in bullous pemphigoid and other autoimmune bullous diseases

Retrospective analysis of the forty-six patients with bullous pemphigoid followed-up in our clinic

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