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Rituximab as first-line treatment of pemphigus
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
The Lancet
Yayıncı:
Elsevier
Yazar(lar) :
Enno Schmidt
Yıl :
2017
Enno Schmidt
Yayıncı Web Sitesi
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Kaydet Alıntıla
DOI : 10.1016/s0140-6736(17)30787-0
ISSN: 0140-6736
Sayı: 10083
Cilt: 389
Sayfa: 1956-1958
Sınıflandırma :
2
Pemphigoid diseases
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
The Lancet
Yayıncı:
Elsevier
Yazar(lar) :
Enno Schmidt , Detlef Zillikens
Yıl :
2013
Enno Schmidt
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Kaydet Alıntıla
Summary Pemphigoid diseases are a group of well defined autoimmune disorders that are characterised by autoantibodies against structural proteins of the dermal–epidermal junction and, clinically, by tense blisters and erosions on skin or mucous membranes close to the skin surface. The most common of these diseases is bullous pemphigoid, which mainly affects older people and the reported incidence of which in Europe has more than doubled in the past decade. Prognosis and treatments vary substantially between the different disorders and, since clinical criteria are usually not sufficient, direct immunofluorescence microscopy of a perilesional biopsy specimen or serological tests are needed for exact diagnosis. In eight pemphigoid diseases the target antigens have been identified molecularly, which has allowed the development of standard diagnostic assays for detection of serum autoantibodies—some of which are commercially available. In this Seminar we discuss the clinical range, diagnostic criteria, diagnostic assay systems, and treatment options for this group of diseases.
DOI : 10.1016/s0140-6736(12)61140-4
ISSN: 0140-6736
Sayı: 9863
Cilt: 381
Sayfa: 320-332
Sınıflandırma :
3
Apoptosis in pemphigus
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
Autoimmunity Reviews
Yayıncı:
Elsevier
Yazar(lar) :
Enno Schmidt , Jens Waschke
Yıl :
2009
Enno Schmidt
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Kaydet Alıntıla
Pemphigus is a severe autoimmune blistering skin disease. The two main forms of pemphigus, pemphigus vulgaris and pemphigus foliaceus, are primarily caused by autoantibodies against the desmosomal cadherins desmoglein 1 and desmoglein 3. The histopathological hallmark is the loss of cell adhesion between neighbouring keratinocytes, a phenomenon called acantholysis. Various underlying mechanisms of acantholysis have been described including apoptosis. The role of apoptosis in pemphigus pathogenesis is unclear at present and the focus of this review. While characteristic signs of apoptosis can be found in lesional patient skin these can be missing in very early lesions of macroscopically perilesional skin where acantholysis is detected by light microscopy. Under experimental conditions, activation of apoptotic signalling can be induced by pemphigus IgG as well as by pemphigus serum which contains large amounts of Fas ligand. Studies using caspase inhibitors which were effective to block acantholysis indicate that the apoptotic machinery contributes to cell dissociation. However, acantholysis can occur in the absence of apoptosis which demonstrates that apoptosis is not strictly required for skin blistering in pemphigus. Taken together, signalling events leading to apoptosis including activation of executioner caspases are not required but may participate in the cellular response to pemphigus autoantibodies leading to acantholysis.
DOI : 10.1016/j.autrev.2009.01.011
ISSN: 1568-9972
Sayı: 7
Cilt: 8
Sayfa: 533-537
Sınıflandırma :
Apoptoz %61
4
Modern diagnosis of autoimmune blistering skin diseases
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
Autoimmunity Reviews
Yayıncı:
Elsevier
Yazar(lar) :
Enno Schmidt , Detlef Zillikens
Yıl :
2010
Enno Schmidt
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
The diagnostic gold standard of autoimmune bullous diseases is the detection of autoantibodies in skin or mucous membranes by direct immunofluorescence microscopy of a perilesional biopsy. The molecular characterisation of several target antigens within the last 10 years has, however, fostered the development of sensitive and specific diagnostic tools that allow the serological diagnosis in about 90% of patients. Based on the recombinant immunodominant portions of the target antigens, ELISA systems are commercially available for the detection of circulating antibodies against desmoglein 1, desmoglein 3, envoplakin, BP180, and BP230. Autoantibodies against the soluble ectodomain of BP180 (LAD-1), laminin 332, type VII collagen, and most recently, laminin γ1 can be detected by Western blotting with recombinant or cell-derived forms of these proteins. The definite differentiation between the various immunobullous disorders that comprise about a dozen entities is increasingly important since more diverse treatment options are employed. Exact diagnosis is also pivotal for the prognosis, since some autoimmune bullous diseases may indicate an underlying tumor. Association with a malignancy has been shown in paraneoplastic pemphigus (in 100%) and anti-laminin 332 mucous pemphigoid (in 25%) In pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, autoantibodies to desmoglein 3, desmoglein 1, and BP180, respectively, have been shown to correlate with the disease activity. The detection of serum autoantibodies during the course of the disease may thus be helpful in guiding treatment decisions in these patients.
DOI : 10.1016/j.autrev.2010.08.007
ISSN: 1568-9972
Sayı: 2
Cilt: 10
Sayfa: 84-89
Sınıflandırma :
5
Diagnosis of Autoimmune Blistering Diseases
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak 1:
Directory of Open Access Journals (DOAJ)
Kaynak 2:
PubMed
Dergi:
Frontiers in Medicine
Yazar(lar) :
Mareike Witte , Detlef Zillikens , Enno Schmidt
Yıl :
2018
Enno Schmidt
Piri Tam Metin Bulucu
Kaydet Alıntıla
Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Circulating antibodies can be detected via indirect immunofluorescence (IIF) microscopy of different tissue substrates including human skin, monkey esophagus, and more recently, recombinant forms of the different target antigens. Latter are also employed in various commercial ELISA systems and by immunoblotting in in-house assays available in specialized laboratories. ELISA systems are also particularly valuable for monitoring of the disease activity during the disease course which can be helpful for treatment decisions. Exact diagnosis is essential for both treatment and prognosis, since some AIBD are associated with malign tumors such as paraneoplastic pemphigus and anti-laminin 332 mucous membrane pemphigoid. This review presents clinical and immunopathological features of AIBD for the state-of the art diagnosis of these disorders.
DOI : 10.3389/fmed.2018.00296
Anahtar Kelimeler : autoantibody, biochip, immunofluorescence, ELISA, pemphigus, pemphigoid
ISSN: 2296-858X
Cilt: 5
Sınıflandırma :
6
Diagnosis of autoimmune bullous diseases.
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
PubMed
Dergi:
J Dtsch Dermatol Ges
Yazar(lar) :
Nina van Beek , Detlef Zillikens , Enno Schmidt
Yıl :
2018
Enno Schmidt
Yayıncı Web Sitesi
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Kaydet Alıntıla
Autoimmune bullous disorders (AIBDs) are a heterogeneous group of rare diseases clinically characterized by erosions and/or blisters on the skin and mucous membranes. AIBDs can be categorized into two groups: pemphigus diseases, characterized by intraepidermal blistering and autoantibodies against desmosomal proteins such as desmoglein (Dsg) 1, Dsg3, members of the plakin family, and subepidermal AIBDs, comprised of pemphigoid diseases and dermatitis herpetiformis. Autoantibodies in dermatitis herpetiformis target transglutaminases 2 and 3, while in pemphigoid disease, autoantibodies are directed against structural proteins of the dermal-epidermal junction. Although analysis of a perilesional biopsy with direct immunofluorescence (IF) microscopy is still the diagnostic gold standard, several assays have become widely available that allow serological diagnosis in the majority of patients. Standard serological diagnosis includes indirect IF on monkey esophagus and salt-split human skin. Assays to further characterize autoantibody specificity include ELISA systems based on recombinant forms of the immunodominant regions of the target antigens as well as multivariant indirect IF microscopy tests with several miniature substrates. These serological assays are complemented by various in-house assays using immunoblotting and ELISA, which are only available in specialized laboratories. Here we review new developments in the diagnosis of AIBDs and describe state-of-the-art diagnostic procedures for this group of diseases.
DOI : 10.1111/ddg.13637
Sınıflandırma :
7
Bullöse Autoimmundermatosen
Dergi Makalesi

Dergi Makalesi
Kaynak :
Springer Link
Dergi:
Der Hautarzt
Yazar(lar) :
Enno Schmidt , Rüdiger Eming
Konu :
Dermatology
Yıl :
2019
Enno Schmidt
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DOI : 10.1007/s00105-019-4391-y
ISSN: 0017-8470 1432-1173
Sayı: 4
Cilt: 70
Sayfa: 234-235
8
Immunoadsorption in dermatology
Dergi Makalesi

Dergi Makalesi
Kaynak 1:
Springer Link
Kaynak 2:
PubMed
Dergi:
Archives of Dermatological Research
Yazar(lar) :
Enno Schmidt , Detlef Zillikens
Konu :
Dermatology, General Medicine
Yıl :
2010
Enno Schmidt
Tam Metin
Kaydet Alıntıla
Immunoadsorption (IA), also termed immunoapheresis, has been established as effective and specific tool advantageous to plasmapheresis to remove immunoglobulin and immune complexes and in cytapheresis, immune cells from the circulation. IA was successfully used in various autoantibody-mediated diseases, e.g. acquired hemophilia, myasthenia gravis, dilated cardiomyopathy, and Guillain–Barré syndrome. In dermatology, IA has been applied as an effective adjuvant treatment for autoimmune bullous diseases. Autoimmune blistering disorders are a heterogeneous group of diseases that are associated with autoantibodies to desmosomal (pemphigus group) and basal membrane zone proteins (pemphigoid group, epidermolysis bullosa acquisita). Because the pathogenic relevance of autoantibodies was clearly demonstrated in the majority of these disorders, removal of autoantibodies, therefore, appears to be a rational therapeutic approach for these patients. IA has been shown to effectively lower the autoantibody levels and leads to rapid clinical responses in patients with immunobullous disorders. Here, clinical effects and adverse events of IA in more than 50 reported patients with autoimmune blistering disorders are reviewed. In addition, an overview of the available IA systems and treatment protocols is provided and guidelines of a recent consensus of German, Austrian, and Swiss experts for the use of IA in autoimmune bullous diseases are summarized.
DOI : 10.1007/s00403-009-1024-9
Anahtar Kelimeler : Autoantibody, Pemphigoid, Immunoapheresis, Pemphigus, Treatment
ISSN: 0340-3696 1432-069X
Sayı: 4
Cilt: 302
Sayfa: 241-253
9
Autoimmune Bullous Diseases
Diğer

Diğer
Kaynak :
Wiley Online Library
Dergi:
Harper's Textbook of Pediatric Dermatology
Yazar(lar) :
Nina Beek , Enno Schmidt
Yıl :
2019
Enno Schmidt
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Kaydet Alıntıla
Autoimmune blistering diseases (AIBDs) are characterized by tissue‐bound and circulating autoantibodies against proteins of epidermal/epithelial desmosomes in pemphigus diseases and proteins of the dermal–epidermal junction in pemphigoid disorders. AIBDs are difficult to distinguish based on clinical presentation alone, in particular in infants and children. Modern diagnosis of AIBDs depends on the clinical picture, direct immunofluorescence microscopy and detection of serum autoantibodies. Treatment of childhood AIBDs is challenging due to the lack of controlled clinical trials and involves a multidisciplinary approach including dermatologists, paediatric dermatologists and paediatricians. Here, essential epidemiological, clinical and immunopathological findings as well as treatment regimens in pemphigus and pemphigoid diseases in a paediatric population are presented.
DOI : 10.1002/9781119142812.ch74
ISBN:9781119142195
Sayfa: 868-897
10
85-year-old male with cutaneous blisters : Preparation for the medical specialist examination: Part 25.
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
PubMed
Dergi:
Hautarzt
Yazar(lar) :
Mareike Witte , Enno Schmidt , Ralf Ludwig
Yıl :
2018
Enno Schmidt
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
DOI : 10.1007/s00105-018-4291-6
Sınıflandırma :
11
Diagnostik blasenbildender Autoimmundermatosen.
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
PubMed
Dergi:
J Dtsch Dermatol Ges
Yazar(lar) :
Nina van Beek , Detlef Zillikens , Enno Schmidt
Yıl :
2018
Enno Schmidt
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
Blasenbildende Autoimmundermatosen (BAIDs) sind eine heterogene Gruppe seltener Erkrankungen, die klinisch durch Erosionen und/oder Blasen an Haut und Schleimhäuten charakterisiert sind. BAIDs können in zwei Gruppen eingeteilt werden: Pemphigus-Erkrankungen, die durch intraepidermale Blasenbildung und Autoantikörper gegen desmosomale Proteine wie Desmoglein (Dsg) 1, Dsg3 und Mitglieder der Plakin-Familie charakterisiert sind, sowie subepidermale BAIDs, die Pemphigoid-Erkrankungen und die Dermatitis herpetiformis umfassen. Bei der Dermatitis herpetiformis greifen die Autoantikörper die Transglutaminasen 2 und 3 an, während sie bei Pemphigoid-Erkrankungen gegen Strukturproteine der dermoepidermalen Junktionszone gerichtet sind. Die Analyse einer periläsionalen Biopsie mittels direkter Immunfluoreszenzmikroskopie (IF-Mikroskopie) stellt zwar nach wie vor den diagnostischen Goldstandard dar, mittlerweile sind jedoch verschiedene Tests allgemein verfügbar, die eine serologische Diagnosefindung für den Großteil der Patienten ermöglichen. Zur serologischen Standarddiagnostik gehört die indirekte IF auf Affenösophagus und NaCl-separierter Spalthaut. Die Spezifität der Autoantikörper kann mit ELISA-Systemen, die auf rekombinanten Formen der immundominanten Regionen der Zielantigene basieren, sowie indirekter IF-Mikroskopie anhand multivarianter Tests mit mehreren multivariate näher charakterisiert werden. Diese serologischen Tests werden durch verschiedene hauseigene Immunoblotting- und ELISA-Systeme ergänzt, die nur in spezialisierten Laboratorien verfügbar sind. In diesem Review geben wir einen Überblick über neue Entwicklungen bei der Diagnose von BAIDs und beschreiben moderne Diagnoseverfahren für diese Krankheitsgruppe.
DOI : 10.1111/ddg.13637_g
Sınıflandırma :
Hukuk %100
Ticaret Hukuku %100
12
Targeting IgE Antibodies by Immunoadsorption in Atopic Dermatitis
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Frontiers in Immunology
Yazar(lar) :
Michael Kasperkiewicz , Enno Schmidt , Enno Schmidt , Ralf J. Ludwig , Ralf J. Ludwig , Detlef Zillikens , Detlef Zillikens
Yıl :
2018
Enno Schmidt Enno Schmidt
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
One major hallmark of atopic dermatitis (AD) is the elevated level of total serum IgE, which has been reported to be partly of the autoreactive type in a subset of patients. Immunoadsorption (IA) has been successfully applied in various classical autoantibody-mediated diseases such as pemphigus. Recent reports proposed the use of IA also for patients with severe AD and high total serum IgE levels. In this mini-review, we summarize the current knowledge about this novel treatment approach for AD and briefly discuss the so far incompletely known role of autoreactive IgE as potential target of IA therapy in this common inflammatory skin disorder.
DOI : 10.3389/fimmu.2018.00254
Anahtar Kelimeler : atopic dermatitis, autoantibody, immunoadsorption, inflammation
ISSN: 1664-3224
Cilt: 9
Sınıflandırma :
13
Forschen für die Praxis: Diagnostik subepidermal blasenbildender Autoimmundermatosen
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Journal der Deutschen Dermatologischen Gesellschaft
Yazar(lar) :
Enno Schmidt , Detlef Zillikens
Konu :
Dermatology
Yıl :
2009
Enno Schmidt
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
DOI : 10.1111/j.1610-0387.2008.06974_supp.x
ISSN: 1610-0379
Sayı: 4
Cilt: 7
Sayfa: 296-300
14
Research in practice: diagnosis of subepidermal autoimmune bullous disorders
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Journal der Deutschen Dermatologischen Gesellschaft
Yazar(lar) :
Enno Schmidt , Detlef Zillikens
Konu :
Dermatology
Yıl :
2009
Enno Schmidt
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Subepidermal autoimmune bullous disorders are a heterogeneous group of diseases that are associated with autoantibodies to hemidesmosomal proteins (pemphigoid group, epidermolysis bullosa acquisita) or epidermal/tissue transglutaminase (dermatitis herpetiformis). Characterization of the target antigens has led to the description of novel entities such as anti‐p200 pemphigoid and has greatly facilitated the diagnosis of these diseases. Precise identification of target antigens is of therapeutic relevance since treatment options may greatly differ between these disorders. By the use of various in vitro and in vivo systems, the pathogenic relevance of autoantibodies to different hemidesmosomal constituents, including BP180 (type XVII collagen), laminin 332 (laminin 5), α6β4 integrin, and type VII collagen has been demonstrated. The translation of this basic scientific work has subsequently led to the development of specific autoantibody detection systems that are not only of diagnostic use but also allow the monitoring of circulating autoantibody levels during the course of the disease and thus are helpful in guiding treatment decisions in these patients.
DOI : 10.1111/j.1610-0387.2008.06974.x
ISSN: 1610-0379
Sayı: 4
Cilt: 7
Sayfa: 296-300
15
Resolution in bullous pemphigoid
Dergi Makalesi

Dergi Makalesi
Kaynak :
Springer Link
Dergi:
Seminars in Immunopathology
Yazar(lar) :
Christian D. Sadik , Enno Schmidt
Konu :
Immunology, Immunology and Allergy
Yıl :
2019
Enno Schmidt
Yayıncı Web Sitesi
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Kaydet Alıntıla
Pemphigoid 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 investigation. Pemphigoid disease patients, particularly bullous pemphigoid patients, who are predominantly above 75 years of age, often succumb to the side effects of the immunosuppressive therapeutics nowadays still required to suppress the disease. Pemphigoid disease patients may therefore represent a group of patients benefiting most substantially from the introduction of non-immunosuppressive, proresolving therapeutics into the treatment regimens for their disease.
DOI : 10.1007/s00281-019-00759-y
Anahtar Kelimeler : Autoimmunity, Bullous pemphigoid, Pathophysiology, IL-17, Complement, Blistering, Resolution
ISSN: 1863-2297 1863-2300
Sayı: 6
Cilt: 41
Sayfa: 645-654
16
Pemphigus
Dergi Makalesi

Dergi Makalesi
Kaynak 1:
Science Direct
Kaynak 2:
Springer Link
Dergi:
The Lancet
Yayıncı:
Elsevier
Yazar(lar) :
Enno Schmidt , Michael Kasperkiewicz , Pascal Joly
Yıl :
2019
Enno Schmidt
Tam Metin
Kaydet Alıntıla
Summary Pemphigus consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly directed against two desmosomal adhesion proteins, desmoglein (Dsg)1 and Dsg3 (also known as DG1 and DG3), which are present in the skin and surface-close mucosae. The binding of autoantibodies to Dsg proteins induces a separation of neighbouring keratinocytes, in a process known as acantholysis. The two main pemphigus variants are pemphigus vulgaris, which often originates with painful oral erosions, and pemphigus foliaceus, which is characterised by exclusive skin lesions. Pemphigus is diagnosed on the basis of either IgG or complement component 3 deposits (or both) at the keratinocyte cell membrane, detected by direct immunofluorescence microscopy of a perilesional biopsy, with serum anti-Dsg1 or anti-Dsg3 antibodies (or both) detected by ELISA. Corticosteroids are the therapeutic mainstay, which have recently been complemented by the anti-CD20 antibody rituximab in moderate and severe disease. Rituximab induces complete remission off therapy in 90% of patients, despite rapid tapering of corticosteroids, thus allowing for a major corticosteroid-sparing effect and a halved number of adverse events related to corticosteroids.
DOI : 10.1016/s0140-6736(19)31778-7
ISSN: 0140-6736
Sayı: 10201
Cilt: 394
Sayfa: 882-894
Sınıflandırma :
17
Current therapy of the pemphigus group
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
Clinics in Dermatology
Yayıncı:
Elsevier
Yazar(lar) :
Michael Kasperkiewicz , Enno Schmidt , Detlef Zillikens
Yıl :
2012
Enno Schmidt
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Treatment of pemphigus patients is still challenging and, in some cases, conventional therapy with systemic corticosteroids in combination with adjuvant corticosteroid-sparing immunosuppressive drugs is not sufficient to induce clinical remission. More recently, high-dose intravenous immunoglobulins, immunoadsorption, and the monoclonal anti-CD20 antibody, rituximab, have been established as additional successful therapeutic options. This contribution covers both conventional therapies and most current treatment strategies for pemphigus.
DOI : 10.1016/j.clindermatol.2011.03.014
ISSN: 0738-081X
Sayı: 1
Cilt: 30
Sayfa: 84-94
Sınıflandırma :
18
85/m mit Blasen am gesamten Integument
Dergi Makalesi

Dergi Makalesi
Kaynak :
Springer Link
Dergi:
Der Hautarzt
Yazar(lar) :
Mareike Witte , Enno Schmidt , Ralf Ludwig
Konu :
Dermatology
Yıl :
2018
Enno Schmidt
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
DOI : 10.1007/s00105-018-4291-6
Anahtar Kelimeler : Bullöses Pemphigoid, Blasenbildende Autoimmundermatose, BP180, BP230, Immunfluoreszenz
ISSN: 0017-8470 1432-1173
Sayı: S2
Cilt: 69
Sayfa: 183-188
19
Structural proteins of the dermal-epidermal junction targeted by autoantibodies in pemphigoid diseases
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Experimental Dermatology
Yazar(lar) :
Stephanie Goletz , Detlef Zillikens , Enno Schmidt
Konu :
Biochemistry, Molecular Biology, Dermatology
Yıl :
2017
Enno Schmidt
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
The dermal‐epidermal junction consists of a network of several interacting structural proteins that strengthen adhesion and mediate signalling events. This structural network consists of hemidesmosomal‐anchoring filament complexes connecting the basal keratinocytes to the basement membrane. The anchoring filaments in turn interact with the anchoring fibrils to attach the basement membrane to the underlying dermis. Several of these structural proteins are recognized by autoantibodies in pemphigoid diseases, a heterogeneous group of clinically and immunopathologically diverse entities. Targeted proteins include the two intracellular plakins, plectin isoform 1a and BP230 (also called bullous pemphigoid antigen (BPAG) 1 isoform e (BPAG1e)). Plectin 1a and BP230 are connected to the intermediate filaments and to the cell surface receptor α6β4 integrin, which in turn is connected to laminin 332, a component of the anchoring filaments. Further essential adhesion proteins are BP180, a transmembrane protein, laminin γ1 and type VII collagen. Latter protein is the major constituent of the anchoring fibrils. Mutations in the corresponding genes of these adhesion molecules lead to inherited epidermolysis bullosa emphasizing the importance of these proteins for the integrity of the dermal‐epidermal junction. This review will provide an overview on the structure and function of the proteins situated in the dermal‐epidermal junction targeted by autoantibodies.
DOI : 10.1111/exd.13446
ISSN: 0906-6705
Sayı: 12
Cilt: 26
Sayfa: 1154-1162
20
Rituximab in Severe Pemphigus
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Annals of the New York Academy of Sciences
Yazar(lar) :
Enno Schmidt , Matthias Goebeler , Detlef Zillikens
Konu :
General Biochemistry, Genetics and Molecular Biology, History and Philosophy of Science
Yıl :
2009
Enno Schmidt
Yayıncı Web Sitesi
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Kaydet Alıntıla
Pemphigus is a life‐threatening autoimmune bullous disorder that requires long‐term treatment with systemic corticosteroids mostly in conjunction with further immunosuppressants. Rituximab has opened a new avenue for severe and refractory pemphigus patients. In 95% of the 136 pemphigus patients reported to date in the English, French, and German literature, at least a partial remission has been achieved. In about two‐thirds of communicated cases, application of rituximab resulted in the healing of all lesions. A controlled prospective study, however, has only recently being initiated. In pemphigus, rituximab is aimed at resetting the immune system to a condition in which autoreactive B lymphocytes are eliminated or sufficiently controlled. Here, we review mechanisms of action, different treatment protocols, clinical responses, and the safety profile of rituximab.
DOI : 10.1111/j.1749-6632.2009.04744.x
ISSN: 0077-8923
Sayı: 1
Cilt: 1173
Sayfa: 683-691
Sınıflandırma :
21
Rituximab in Treatment-Resistant Autoimmune Blistering Skin Disorders
Dergi Makalesi

Dergi Makalesi
Kaynak :
Springer Link
Dergi:
Clinical Reviews in Allergy & Immunology
Yazar(lar) :
Enno Schmidt , Eva-Bettina Bröcker , Matthias Goebeler
Konu :
General Medicine, Immunology and Allergy
Yıl :
2007
Enno Schmidt
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Kaydet Alıntıla
Autoimmune blistering diseases are associated with autoantibodies to desmosomal (pemphigus group) or hemidesmosomal proteins (autoimmune subepidermal blistering disorders) that are essential for the structural integrity of the epidermis and dermoepidermal junction. Treatment is usually based on systemic glucocorticosteroids, which are often combined with additional immunosuppressants such as azathioprine and mycophenolate mofetil or immunomodulators including dapsone, antibiotics, and intravenous immunoglobulins. These interventions are sometimes not sufficient to induce remission and/or may be associated with intolerable adverse events. In such situations, the anti-CD20 antibody rituximab has been successfully applied in recent years. Rituximab transitorily depletes CD20-positive B lymphocytes from the circulation. It has been employed in more than 1 million patients with CD20-positive non-Hodgkin’s lymphoma and severe side effects were only rarely observed. Subsequently, the B cell-modulating effect of rituximab has encouraged its use in a variety of autoimmune diseases, including more than 40 patients with pemphigus. In addition, a few patients with bullous pemphigoid, mucous membrane pemphigoid, and epidermolysis bullosa acquisita have received rituximab. In the majority of these patients, clinical remission was induced; however, serious adverse events were considerable higher compared to both patients with non-Hodgkin’s lymphoma or nonbullous autoimmune disorders like lupus erythematosus, dermatomyositis, and rheumatoid arthritis.
DOI : 10.1007/s12016-007-8021-6
Anahtar Kelimeler : Autoantibody, B lymphocyte, Bullous pemphigoid, Epidermolysis bullosa acquisita, MabThera™, Pemphigus, Rituxan™, Therapy
ISSN: 1080-0549 1559-0267
Sayı: 1
Cilt: 34
Sayfa: 56-64
22
Covid‐19 pandemic and the skin
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
International Journal of Dermatology
Yazar(lar) :
Kossara Drenovska , Enno Schmidt , Snejina Vassileva
Konu :
Dermatology
Yıl :
2020
Enno Schmidt
Yayıncı Web Sitesi
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In the beginning of the COVID‐19 outbreak, skin manifestations, if present, were not paid enough attention. Then, the focus moved toward the impact of the prolonged use of personal protective measures in both healthcare workers and patients. In the meantime, attention is increasingly paid to dermatology as a result of the concern for certain groups of dermatologic patients, including those whose condition may worsen by the thorough disinfection measures and those treated with immunosuppressants or immunomodulators. Following patients with psoriasis on biological therapy, as well as other inflammatory and autoimmune cutaneous disorders such as atopic dermatitis, pemphigus, pemphigoid diseases, and skin cancer provoked the interest of dermatologists. Finally, an intriguing question to the dermatologic society was whether skin changes during COVID‐19 infection exist and what could be their diagnostic or prognostic value. Here, we summarize skin conditions during the COVID‐19 pandemic, patient information, and expert recommendations and give an overview about the registries launched to document skin changes during COVID‐19, as well as details about certain patient groups infected with SARS‐CoV‐2, for example, psoriasis, atopic dermatitis, and autoimmune bullous diseases.
DOI : 10.1111/ijd.15189
ISSN: 0011-9059
Sayı: 11
Cilt: 59
Sayfa: 1312-1319
23
Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Case Reports in Dermatology
Yazar(lar) :
Rebecca Mergler , Andreas Kerstan , Enno Schmidt , Matthias Goebeler , Sandrine Benoit
Yıl :
2017
Enno Schmidt
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Kaydet Alıntıla
Pemphigus vegetans (PVeg) is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment. Diagnosis is confirmed by immunofluorescence and detection of anti-desmoglein (Dsg) 3 and/or anti-Dsg1 antibodies. We herein report an unusual manifestation of PVeg. At the time of first presentation, lesions were restricted to the right ring finger’s tip. Although mucous membranes were initially not affected, high levels of anti-Dsg3 antibodies were detected while anti-Dsg1 and anti-desmocollin (Dsc) 1, 2, and 3 antibodies were absent. To compare our immunological findings with previous reports, all accessible Anglophone literature published since December 1988 was evaluated. We identified 52 patients suffering from PVeg, 7 of these showed anti-Dsg3 antibodies without any mucous membrane involvement. Notably, the detection of anti-Dsg1 and anti-Dsg3 antibodies does not necessarily correlate with the involvement of skin and/or mucous membranes. This might be due to more specific and complex antibody constellations in nonclassical or atypical pemphigus.
DOI : 10.1159/000468919
Anahtar Kelimeler : Desmocollin, Desmoglein, Pemphigus, Pemphigus vegetans, Review
ISSN: 1662-6567
Cilt: 9
Sayı: 1
Sayfa: 121 - 130
Sınıflandırma :
24
Anti-p200 pemphigoid
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
Journal of the American Academy of Dermatology
Yayıncı:
Elsevier
Yazar(lar) :
Stephanie Goletz , Takashi Hashimoto , Detlef Zillikens , Enno Schmidt
Yıl :
2014
Enno Schmidt
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Anti-p200 pemphigoid is a rare subepidermal blistering skin disease. Patients' autoantibodies label the dermal side of 1 mol/L NaCl-split human skin by indirect immunofluorescence microscopy and recognize a 200-kd protein by immunoblotting of human dermal extract. Clinically, anti-p200 pemphigoid is characterized by tense blisters and vesicles, erosions, and urticarial plaques, closely resembling bullous pemphigoid and the inflammatory variant of epidermolysis bullosa acquisita. Recently, 90% of anti-p200 pemphigoid sera were shown to recognize laminin γ1. The C-terminus of laminin γ1 was identified as an immunodominant region and in its recombinant form was used by immunoblotting and enzyme-linked immunosorbent assay for the serologic diagnosis of this disease. Subsequent ex vivo and in vivo studies were, however, unable to show pathogenic activity of antilaminin γ1 antibodies. Both patients' sera and sera depleted from antilaminin γ1 antibodies induced subepidermal splitting in an ex vivo model of autoantibody-mediated leukocyte-dependent neutrophil activation. Antilaminin γ1 antibodies appear to be useful biomarkers that will further facilitate the diagnosis of anti-p200 pemphigoid. The true identity of the pathogenetically relevant autoantigen of this disease, which may either be a yet unknown isoform of laminin γ1 or even another 200-kd protein of the dermoepidermal junction, still needs to be elucidated.
DOI : 10.1016/j.jaad.2014.02.036
Anahtar Kelimeler : anti-p200 pemphigoid, autoantibody, autoantigen, laminin γ1, p200 antigen, subepidermal blistering skin disease, DES dermoepidermal separation, dermoepidermal separation, IF immunofluorescence, immunofluorescence
ISSN: 0190-9622
Sayı: 1
Cilt: 71
Sayfa: 185-191
Sınıflandırma :
25
Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Frontiers in Medicine
Yazar(lar) :
Stanislav Khil'chenko , Stanislav Khil'chenko , Katharina Boch , Katharina Boch , Nina van Beek , Nina van Beek , Artem Vorobyev , Artem Vorobyev , Detlef Zillikens , Detlef Zillikens , Enno Schmidt , Enno Schmidt , Enno Schmidt , Ralf J. Ludwig , Ralf J. Ludwig
Yıl :
2020
Enno Schmidt Enno Schmidt Enno Schmidt
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
Pemphigus and pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes, which are caused by autoantibodies targeting structural proteins of the skin. In other autoimmune diseases, a high prevalence of primary antibody deficiencies was noted. Conversely, a high prevalence of autoimmune diseases is reported in patients with primary antibody deficiencies. With the exception of one study, pointing toward a decrease of IgG in pemphigus patients, with a relative enrichment of IgG4, serum immunoglobulin (Ig) concentrations had not been studied in pemphigus and pemphigoid. Hence, we here aimed to investigate serum concentrations of IgM, IgA, IgG, and IgG1–4 in pemphigus and pemphigoid patients, as well as in healthy controls. Serum Ig concentrations were determined by ELISA in 105 healthy controls, 100 pemphigus vulgaris (PV), 100 pemphigus foliaceus, 99 bullous pemphigoid (BP), and 55 linear IgA bullous dermatosis (LAD) patients. In healthy controls, age had a significant impact on Ig serum concentrations: In controls at ages of 69 years or older, IgM and IgG were decreased, while all other Ig, except IgA and IgG4, were increased. When stratified by sex, lower IgM concentrations were observed in males. When corrected for age and/or sex, and compared to controls, an increase in serum IgA was noted in LAD. In almost all patient cohorts, an increase in IgG1 and IgG4 was observed, while a decrease in IgG2 or IgG3 was seen in BP or PV patients. This points toward a possible association of BP with IgG2 deficiency and warrants evaluation of IgG2 in BP patients prior to immunosuppressive therapy.
DOI : 10.3389/fmed.2020.00472
Anahtar Kelimeler : pemphigus, pemphigoid, immunoglobulin
ISSN: 2296-858X
Cilt: 7
Sınıflandırma :
26
Blasenbildende Autoimmundermatosen
Dergi Makalesi

Dergi Makalesi
Kaynak :
Springer Link
Dergi:
hautnah dermatologie
Yazar(lar) :
Osman Kaan Yilmaz , Detlef Zillikens , Enno Schmidt
Yıl :
2021
Enno Schmidt
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DOI : 10.1007/s15012-021-6653-5
ISSN: 0938-0221 2196-6451
Sayı: 3
Cilt: 37
Sayfa: 46-57
27
Immunoadsorption of Desmoglein-3-Specific IgG Abolishes the Blister-Inducing Capacity of Pemphigus Vulgaris IgG in Neonatal Mice
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
Directory of Open Access Journals (DOAJ)
Dergi:
Frontiers in Immunology
Yazar(lar) :
Maxi Hofrichter , Jenny Dworschak , Shirin Emtenani , Jana Langenhan , Fanny Weiß , Lars Komorowski , Detlef Zillikens , Winfried Stöcker , Christian Probst , Enno Schmidt , Enno Schmidt , Stephanie Goletz
Yıl :
2018
Enno Schmidt Enno Schmidt
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Kaydet Alıntıla
Pemphigus vulgaris (PV) is a potentially life-threatening autoimmune blistering disease which is associated with autoantibodies directed against two desmosomal proteins, desmoglein (Dsg) 3 and 1. Treatment of PV is rather challenging and relies on the long-term use of systemic corticosteroids and additional immunosuppressants. More recently, autoantibody-depleting therapies such as rituximab, high-dose intravenous immunoglobulins, and immunoadsorption were shown to be valuable treatment options in PV. Specific removal of pathogenic autoantibodies would further increase efficacy and usability of immunoadsorption. Here, we tested the capacity of our recently developed prototypic Dsg1- and Dsg3-specific adsorbers to remove circulating pathogenic autoantibodies from three different PV patients. The pathogenic potential of the Dsg3/1-depleted IgG fractions and the anti-Dsg3-specific IgG was explored in two different in vitro assays based on cultured human keratinocytes, the desmosome degradation assay and the dispase-based dissociation assay. In addition, the neonatal mouse model of PV was used. In both in vitro assays, no difference between the pathogenic effect of total PV IgG and anti-Dsg3-specific IgG was seen, while Dsg3/1-depleted and control IgG were not pathogenic. For the samples of all 3 PV patients, depletion of anti-Dsg3/1 IgG resulted in a complete loss of pathogenicity when injected into neonatal mice. In contrast, injection of anti-Dsg3-specific IgG, eluted from the column, induced gross blistering in the mice. Our data clearly show that anti-Dsg3-specific IgG alone is pathogenic in vitro and in vivo, whereas Dsg3/1-depletion results in a complete loss of pathogenicity. Furthermore, our data suggest that Dsg-specific adsorption may be a suitable therapeutic modality to efficiently reduce pathogenic autoantibodies in patients with severe PV.
DOI : 10.3389/fimmu.2018.01935
Anahtar Kelimeler : acantholysis, autoantibody, desmoglein, desmosome, immunoadsorption, pemphigus
ISSN: 1664-3224
Cilt: 9
Sınıflandırma :
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