Activation of blood coagulation in bullous pemphigoid: role of eosinophils, and local and systemic implications

Marzano, Angelo Valerio; Tedeschi, A.; Fanoni, Daniele; Bonanni, Erika; Venegoni, Luigia; Berti, Emilio; Cugno, Massimo

doi:10.1111/j.1365-2133.2008.08880.x

Cited by 79 publications

(116 citation statements)

References 24 publications

Supporting

Mentioning

100

Contrasting

Unclassified

Order By: Relevance

“…In fact Fujii and co-workers (48) found that the coagulation biomarkers that we routinely measure in patients with CU reflect an activation of the coagulation cascade that in CU occurs extravascularly, which may explain the low prevalence of thrombosis in this disease. In contrast, the activation of coagulation in patients with other skin diseases such as BP is associated with an increased thrombotic risk (9,88). Chronic urticaria is frequently characterized by the presence of elevated levels of plasma markers of thrombin generation and fibrinolysis during severe exacerbations of the disease (37,38,47), which can be due to the expression of TF by activated eosinophils (22).…”

Section: Discussionmentioning

confidence: 99%

Chronic urticaria and coagulation: pathophysiological and clinical aspects

Tedeschi

Kolkhir

Asero³

et al. 2014

Allergy

Self Cite

View full text Add to dashboard Cite

Chronic urticaria (CU) is a widespread skin disease, characterized by the recurrence of transient wheals and itch for more than 6 weeks. Besides autoimmune mechanisms, coagulation factors, in particular tissue factor and thrombin, might also participate in the disease pathophysiology. Tissue factor expressed by eosinophils can induce activation of blood coagulation generating thrombin which in turn can increase vascular permeability both directly, acting on endothelial cells, and indirectly, inducing degranulation of mast cells with release of histamine, as demonstrated in experimental models. D-dimer, a fibrin degradation product, generated following activation of the coagulation cascade and fibrinolysis, has been found to be increased during urticaria exacerbations; moreover, it has been proposed as a biomarker of severity and resistance to H1-antihistamines in CU patients. The possible role of coagulation in CU is also supported by case reports, case series and a small controlled study showing the efficacy of anticoagulant therapy in this disease. The purpose of this review was to summarize the available data on the possible contribution of coagulation to the pathophysiology of CU focusing on clinical aspects and possible future therapeutic developments.Chronic urticaria (CU) is a widespread skin disease characterized by the recurrence of transient wheals and itch for more than 6 weeks. It is believed that over 50% of CU cases are accompanied by a deep subcutaneous and/or submucosal edema, called angioedema (AE; 1).It is well known that the characteristic symptoms of urticaria appear following the activation of mast cells (MCs) and basophils in the skin by various stimuli. These cells release several biologically active substances, the most important of which is histamine. Histamine induces vasodilation, increases vascular permeability and stimulates sensory nerve endings. These effects lead to the appearance of erythema, wheals and itch (2). It is supposed that other biologically active substances may have similar effects. These include serotonin, C3a and C5a anaphylatoxins, platelet-activating factor (PAF), neuropeptides, and arachidonic acid metabolites (prostaglandin D2, leukotrienes C4, D4 and E4; 3).Activation and degranulation of basophils and MCs may occur by immunological (formation of immune complexes, complement activation, IgE cross-linking), nonimmunological (pseudoallergy, infection, or direct effect of agents on MCs), or mixed mechanisms. The mechanism and cause of urticaria remain unclear in many patients with CU.In recent years, attention was paid to several important aspects of the disease pathogenesis. It is believed that about 30-50% of patients have CU symptoms associated with autoimmune reactions and synthesis of autoantibodies against IgE (4) and/or the FceRI a-subunit on MCs and basophils (autoimmune/autoreactive urticaria; 5). Furthermore, these patients show an increased frequency of HLA DRB1*04 (DR4) as it occurs in other autoimmune diseases (6). The role of coagulation casc...

show abstract

Section: Discussionmentioning

confidence: 99%

Chronic urticaria and coagulation: pathophysiological and clinical aspects

Tedeschi

Kolkhir

Asero³

et al. 2014

Allergy

Self Cite

View full text Add to dashboard Cite

show abstract

“…Marzano ve ark. yaptıkları bir çalışmada koagülasyon kaskadının aktive olduğunu, hiperkoagülabilitenin inflamasyon, bül formasyonu ve tromboz oluşumuna neden olduğu ve bu durumun hastalık şiddeti ile ilişkili olabildiğini savunmuşlardır 32 . BP'ye eşlik edebilen hastalıklar yaş ile de sıklığında artış olan hastalıklardır, bu nedenle birliktelik rastlantısal olabilir.…”

Section: Türk Dermunclassified

Otoimmün büllü hastalıkların klinik ve demografik özellikleri: 85 hastanın retrospektif analizi

Gül¹,

Kılıç²,

Çakmak³

et al. 2014

TURKDERM

View full text Add to dashboard Cite

ÖzetAmaç: Otoimmün büllü hastalıklar, derideki spesifik antijenlere yönelik dokuda ya da kanda otoantikorların varlığı ile karakterize deri ve/veya mukoza tutulumu ile seyreden hastalıklar grubudur. Çalışmamızın amacı kliniğimize başvuran otoimmün büllü hastalıkların demografik ve klinik özelliklerini değerlendirmektir. Yöntem ve Gereçler: 2003-2011 yılları arasında kliniğimize başvuran klinik, histopatolojik ve immunopatolojik kriterlere dayanılarak otoimmün büllü hastalıklar tanısı konan hastalar retrospektif olarak değerlendirildi. Olgular; cinsiyetleri, yaşları, otoimmün büllü hastalık tanısı, lezyonların başlangıç zamanı ve lokalizasyonu, tutulum yaygınlığı, eşlik eden hastalıklar, hastaların aldığı tedavi, tedaviye yanıtları açısından değerlendirildi. Bulgular: Çalışmamıza toplam 85 hasta alındı. Hastaların 49'u kadın (%57,64), 36'sı erkekti (%42,36). Olguların 57'si (%67,05) pemfigus (P), 24'ü ise (%28,23) pemfigoid grubundaydı. Pemfigus vulgarisli olguların büllöz pemfigoidli olgulara oranı 3,16 olarak saptandı. Lezyonların başlangıç yaşları 22 ile 82 arasında değişiyordu ve kronisitesi 2 hafta ile 120 ay arasında değişiyordu. Olguların takipleri sırasında pemfigus vulgarisli bir olguya mide kanseri tanısı konuldu. Büllöz pemfigoidli üç olguda malinite eşlik ediyordu: Bir olgu kemik metastazlı opere meme karsinomu, bir olgu renal karsinoma ve bir olguda lenfoma mevcut idi. Bir olgu hariç tüm olgularımız klasik tedaviye yanıt verdi. Sonuç: Çalışmamızda en sık pemfigus vulgarise rastladık, ancak pemfigus/büllöz pemfigoid oranını ise daha önceki çalışmalara göre daha düşük saptadık. Ayrıca otoimmün büllü hastalıkların çeşitli hastalıklarla birlikte görülebilme ihtimali nedeniyle detaylı araştırılmaları gerektiğini vurgulamak istiyoruz. (Türk derm 2014; 48: 7-12) Anah tar Ke li me ler: Pemfigus vulgaris, büllöz pemfigoid, otoimmün büllü hastalık Sum maryBackground and Design: Autoimmune bullous dermatoses are a group of diseases characterized by the presence of autoantibodies in tissue or blood against specific cutaneous antigens. The aim of our study was to evaluate the demographical and clinical features of patients with autoimmune bullous diseases who applied to our clinic. Materials and Methods: We retrospectively analyzed the patients who were diagnosed with autoimmune bullous diseases according to clinical, histopathological and immunopathological criteria between the years 2003 and 2011. The subjects were evaluated according to their sex, age, the type of autoimmune bullous disease, the onset and localization of lesions, the extensity of involvement, associated diseases, the treatment they were given and the response to the treatment. Results: A total of 85 cases were included in our study. Fourty-nine (57.64%) patients were female, 36 (42.36%) were male. Fifty-seven cases (67.05%) belonged to pemphigus (P) group and 24 (28.23%) to pemphigoid. The ratio of patients with pemphigus vulgaris to the patients with pemphigoid was found to be 3.16. The age range of the onset of the symptoms was between 22 an...

show abstract

“…These changes were reversible by corticosteroid treatment. [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

View full text Add to dashboard Cite

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

show abstract

Activation of blood coagulation in bullous pemphigoid: role of eosinophils, and local and systemic implications

Cited by 79 publications

References 24 publications

Chronic urticaria and coagulation: pathophysiological and clinical aspects

Chronic urticaria and coagulation: pathophysiological and clinical aspects

Otoimmün büllü hastalıkların klinik ve demografik özellikleri: 85 hastanın retrospektif analizi

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

Contact Info

Product

Resources

About