Bullous pemphigoid induced by IgG targeting type XVII collagen non‐NC16A/NC15A extracellular domains is driven by Fc gamma receptor‐ and complement‐mediated effector mechanisms and is ameliorated by neonatal Fc receptor blockade

Efgartigimod: First Approval

(2022) Young-A Heo   DRUGS

C5aR2 deficiency ameliorates inflammation in murine epidermolysis bullosa acquisita by regulating FcγRIIb expression on neutrophils

(2022) Daniel L. Seiler et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Increased Fibrosis in a Mouse Model of Anti-Laminin 332 Mucous Membrane Pemphigoid Remains Unaltered by Inhibition of Aldehyde Dehydrogenase

(2022) Sabrina Patzelt et al.   Frontiers in Immunology

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology ( EADV )

(2022) L. Borradori et al.   JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY

Insights Into the Pathogenesis of Bullous Pemphigoid: The Role of Complement-Independent Mechanisms

(2022) Connor Cole et al.   Frontiers in Immunology

The relevance of complement in pemphigoid diseases: A critical appraisal

(2022) Cristian Papara et al.   Frontiers in Immunology

Type XVII collagen: Relevance of distinct epitopes, complement-independent effects, and association with neurological disorders in pemphigoid disorders

(2022) Bianca Opelka et al.   Frontiers in Immunology

The Fab region of IgG impairs the internalization pathway of FcRn upon Fc engagement

(2022) Maximilian Brinkhaus et al.   Nature Communications

Differential expression of C5aR1 and C5aR2 in innate and adaptive immune cells located in early skin lesions of bullous pemphigoid patients

(2022) Shirin Emtenani et al.   Frontiers in Immunology

The impact of lesional inflammatory cellular infiltrate on the phenotype of bullous pemphigoid

(2021) Sascha Ständer et al.   JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY

Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open‐label feasibility trial

(2021) M. Goebeler et al.   BRITISH JOURNAL OF DERMATOLOGY

BP180/Collagen XVII: A Molecular View

(2021) Jussi Tuusa et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The pathogeneses of pemphigus and pemphigoid diseases

(2021) Hideyuki Ujiie et al.   JOURNAL OF DERMATOLOGICAL SCIENCE

IgG regulation through FcRn blocking: A novel mechanism for the treatment of myasthenia gravis

(2021) Gil I. Wolfe et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Pemphigus and Pemphigoid: From Disease Mechanisms to Druggable Pathways

(2021) Christoph T. Ellebrecht et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Anti‐neonatal Fc receptor (FcRn) antibody treatment ameliorates experimental epidermolysis bullosa acquisita in mice

(2020) Anika Kasprick et al.   BRITISH JOURNAL OF PHARMACOLOGY

Dissecting the mechanism of action of intravenous immunoglobulin in human autoimmune disease: lessons from therapeutic modalities targeting Fcγ receptors

(2020) Anthony Shock et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Targeting FcRn for immunomodulation: Benefits, risks, and practical considerations

(2020) Hans-Hartmut Peter et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Neonatal Fc receptor in human immunity: Function and role in therapeutic intervention

(2020) Dhavalkumar D. Patel et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Autoantibodies to BPAG1e trigger experimental bullous pemphigoid in mice

(2020) Eiko Makita et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Next-generation Fc receptor–targeting biologics for autoimmune diseases

(2019) Adrian W. Zuercher et al.   AUTOIMMUNITY REVIEWS

Regulatory T-cell deficiency leads to pathogenic bullous pemphigoid antigen 230 autoantibody and autoimmune bullous disease

(2018) Stefanie Haeberle et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

High Expression of Collagen XVII Compensates for its Depletion Induced by Pemphigoid IgG in the Oral Mucosa

(2018) Mayumi Kamaguchi et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Tissue-targeted complement therapeutics

(2018) Stephen Tomlinson et al.   MOLECULAR IMMUNOLOGY

BP180 dysfunction triggers spontaneous skin inflammation in mice

(2018) Yang Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Tissue Destruction in Bullous Pemphigoid Can Be Complement Independent and May Be Mitigated by C5aR2

(2018) Christian M. Karsten et al.   Frontiers in Immunology

IL-17A is functionally relevant and a potential therapeutic target in bullous pemphigoid

(2018) Lenche Chakievska et al.   JOURNAL OF AUTOIMMUNITY

Neonatal Fc receptor antagonist efgartigimod safely and sustainably reduces IgGs in humans

(2018) Peter Ulrichts et al.   JOURNAL OF CLINICAL INVESTIGATION

Fc-binding proteins enhance autoantibody-induced BP180 depletion in pemphigoid

(2018) Hiroaki Iwata et al.   JOURNAL OF PATHOLOGY

In vitro and in vivo models to investigate the pathomechanisms and novel treatments for pemphigoid diseases

(2017) Katja Bieber et al.   EXPERIMENTAL DERMATOLOGY

Structural proteins of the dermal-epidermal junction targeted by autoantibodies in pemphigoid diseases

(2017) Stephanie Goletz et al.   EXPERIMENTAL DERMATOLOGY

A randomized double-blind trial of intravenous immunoglobulin for bullous pemphigoid

(2017) Masayuki Amagai et al.   JOURNAL OF DERMATOLOGICAL SCIENCE

Experimental Laminin 332 Mucous Membrane Pemphigoid Critically Involves C5aR1 and Reflects Clinical and Immunopathological Characteristics of the Human Disease

(2017) Eva Nina Heppe et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Correlation of Serum Levels of IgE Autoantibodies Against BP180 With Bullous Pemphigoid Disease Activity

(2017) Nina van Beek et al.   JAMA Dermatology

BP180 Is Critical in the Autoimmunity of Bullous Pemphigoid

(2017) Yale Liu et al.   Frontiers in Immunology

Epitope-Dependent Pathogenicity of Antibodies Targeting a Major Bullous Pemphigoid Autoantigen Collagen XVII/BP180

(2016) Mayumi Wada et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Treatment of recalcitrant bullous pemphigoid (BP) with a novel protocol: A retrospective study with a 6-year follow-up

(2016) A. Razzaque Ahmed et al.   JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

Mouse and human FcR effector functions

(2015) Pierre Bruhns et al.   IMMUNOLOGICAL REVIEWS

Allelic and copy-number variations of FcγRs affect granulocyte function and susceptibility for autoimmune blistering diseases

(2015) Andreas Recke et al.   JOURNAL OF AUTOIMMUNITY

Deletion of the Major Bullous Pemphigoid Epitope Region of Collagen XVII Induces Blistering, Autoimmunization, and Itching in Mice

(2015) Tiina Hurskainen et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Fcγ Receptors III and IV Mediate Tissue Destruction in a Novel Adult Mouse Model of Bullous Pemphigoid

(2014) Franziska S. Schulze et al.   AMERICAN JOURNAL OF PATHOLOGY

Blister-inducing antibodies target multiple epitopes on collagen VII in mice

(2014) Kinga Csorba et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Passive transfer of collagen XVII-specific antibodies induces sustained blistering disease in adult mice

(2013) Mircea TEODOR Chiriac et al.   Orphanet Journal of Rare Diseases

Diagnosis of oral mucous membrane pemphigoid by means of combined serologic testing

(2013) Taihei Hayakawa et al.   Oral Surgery Oral Medicine Oral Pathology Oral Radiology

Epitope mapping of BP230 leading to a novel enzyme-linked immunosorbent assay for autoantibodies in bullous pemphigoid

(2012) I.M. Blöcker et al.   BRITISH JOURNAL OF DERMATOLOGY

The immunoglobulin, IgG Fc receptor and complement triangle in autoimmune diseases

(2012) Christian M. Karsten et al.   IMMUNOBIOLOGY

Antibodies to Pathogenic Epitopes on Type XVII Collagen Cause Skin Fragility in a Complement-Dependent and -Independent Manner

(2012) K. Natsuga et al.   JOURNAL OF IMMUNOLOGY

Genetic identification and functional validation of FcγRIV as key molecule in autoantibody-induced tissue injury

(2012) Michael Kasperkiewicz et al.   JOURNAL OF PATHOLOGY

Missing the target: Characterization of bullous pemphigoid patients who are negative using the BP180 enzyme-linked immunosorbant assay

(2012) Janet A. Fairley et al.   JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

Pemphigoid diseases

(2012) Enno Schmidt et al.   LANCET

Anti-inflammatory activity of IgG1 mediated by Fc galactosylation and association of FcγRIIB and dectin-1

(2012) Christian M Karsten et al.   NATURE MEDICINE

The Flavonoid Luteolin Inhibits Fcγ-Dependent Respiratory Burst in Granulocytes, but Not Skin Blistering in a New Model of Pemphigoid in Adult Mice

(2012) Eva Oswald et al.   PLoS One

Pathogenicity of Autoantibodies in Anti-p200 Pemphigoid

(2012) Katerina Vafia et al.   PLoS One

Repetitive Immunization Breaks Tolerance to Type XVII Collagen and Leads to Bullous Pemphigoid in Mice

(2011) M. Hirose et al.   JOURNAL OF IMMUNOLOGY

Development of a simple enzyme-linked immunosorbent assay for the detection of autoantibodies in anti-p200 pemphigoid

(2010) S. Groth et al.   BRITISH JOURNAL OF DERMATOLOGY

Human IgG1 Monoclonal Antibody against Human Collagen 17 Noncollagenous 16A Domain Induces Blisters via Complement Activation in Experimental Bullous Pemphigoid Model

(2010) Q. Li et al.   JOURNAL OF IMMUNOLOGY

A Novel Active Mouse Model for Bullous Pemphigoid Targeting Humanized Pathogenic Antigen

(2010) Hideyuki Ujiie et al.   JOURNAL OF IMMUNOLOGY

IgG from Patients with Bullous Pemphigoid Depletes Cultured Keratinocytes of the 180-kDa Bullous Pemphigoid Antigen (Type XVII Collagen) and Weakens Cell Attachment

(2009) Hiroaki Iwata et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

IgE autoantibodies against the intracellular domain of BP180

(2008) S.K. Dresow et al.   BRITISH JOURNAL OF DERMATOLOGY

Subepidermal blistering induced by human autoantibodies to BP180 requires innate immune players in a humanized bullous pemphigoid mouse model

(2008) Zhi Liu et al.   JOURNAL OF AUTOIMMUNITY