Pemphigus: a Comprehensive Review on Pathogenesis, Clinical Presentation and Novel Therapeutic Approaches

The pathogenesis of pemphigus: Controversy vs complexity

(2017) Giovanni Di Zenzo et al.   EXPERIMENTAL DERMATOLOGY

Inhibition of B cell activating factor (BAFF) in the management of systemic lupus erythematosus (SLE)

(2017) William Stohl   Expert Review of Clinical Immunology

Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016

(2017) Enno Schmidt et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Evaluation of JAK3 Biology in Autoimmune Disease Using a Highly Selective, Irreversible JAK3 Inhibitor

(2017) Fiona Elwood et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS

First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial

(2017) Pascal Joly et al.   LANCET

Correlation between IL-36α and IL-17 and Activity of the Disease in Selected Autoimmune Blistering Diseases

(2017) Agnieszka Żebrowska et al.   MEDIATORS OF INFLAMMATION

Th1/Th17-Related Cytokines and Chemokines and Their Implications in the Pathogenesis of Pemphigus Vulgaris

(2017) Rodolfo Pessato Timoteo et al.   MEDIATORS OF INFLAMMATION

Controlled Delivery of Single or Multiple Antigens in Tolerogenic Nanoparticles Using Peptide-Polymer Bioconjugates

(2017) Ryan M. Pearson et al.   MOLECULAR THERAPY

The emerging safety profile of JAK inhibitors in rheumatic disease

(2017) Kevin L. Winthrop   Nature Reviews Rheumatology

Pemphigus

(2017) Michael Kasperkiewicz et al.   Nature Reviews Disease Primers

Different signaling patterns contribute to loss of keratinocyte cohesion dependent on autoantibody profile in pemphigus

(2017) Elias Walter et al.   Scientific Reports

Mechanisms of Disease: Pemphigus and Bullous Pemphigoid

(2016) Christoph M. Hammers et al.   Annual Review of Pathology-Mechanisms of Disease

Monopathogenic vs multipathogenic explanations of pemphigus pathophysiology

(2016) A. Razzaque Ahmed et al.   EXPERIMENTAL DERMATOLOGY

Induction of T regulatory cells by the superagonistic anti-CD28 antibody D665 leads to decreased pathogenic IgG autoantibodies against desmoglein 3 in a HLA-transgenic mouse model of pemphigus vulgaris

(2016) Thomas Schmidt et al.   EXPERIMENTAL DERMATOLOGY

Effect of Macitentan on the Development of New Ischemic Digital Ulcers in Patients With Systemic Sclerosis

(2016) Dinesh Khanna et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Future therapies for pemphigus vulgaris: Rituximab and beyond

(2016) Amy Huang et al.   JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

European Guidelines (S1) on the use of high-dose intravenous immunoglobulin in dermatology

(2016) A.H. Enk et al.   JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY

Low-dose interleukin-2 treatment selectively modulates CD4+ T cell subsets in patients with systemic lupus erythematosus

(2016) Jing He et al.   NATURE MEDICINE

Plasma cells as an innovative target in autoimmune disease with renal manifestations

(2016) Falk Hiepe et al.   Nature Reviews Nephrology

Reengineering chimeric antigen receptor T cells for targeted therapy of autoimmune disease

(2016) C. T. Ellebrecht et al.   SCIENCE

Loss of flotillin expression results in weakened desmosomal adhesion and Pemphigus vulgaris-like localisation of desmoglein-3 in human keratinocytes

(2016) Frauke Völlner et al.   Scientific Reports

Follicular Helper T Cells in Systemic Lupus Erythematosus: Why Should They Be Considered as Interesting Therapeutic Targets?

(2016) Matthieu Sawaf et al.   Journal of Immunology Research

Systemic lupus erythematosus

(2016) Arvind Kaul et al.   Nature Reviews Disease Primers

Increased Frequency of T Follicular Helper Cells and Elevated Interleukin-27 Plasma Levels in Patients with Pemphigus

(2016) Tina Hennerici et al.   PLoS One

Pathogenicity and Epitope Characteristics Do Not Differ in IgG Subclass-Switched Anti-Desmoglein 3 IgG1 and IgG4 Autoantibodies in Pemphigus Vulgaris

(2016) Agnes S. Lo et al.   PLoS One

The proteasome inhibitior bortezomib depletes plasma cells and ameliorates clinical manifestations of refractory systemic lupus erythematosus

(2015) Tobias Alexander et al.   ANNALS OF THE RHEUMATIC DISEASES

Clinical and immunological findings in 104 cases of paraneoplastic pemphigus

(2015) A. Ohzono et al.   BRITISH JOURNAL OF DERMATOLOGY

Effective targeting of primitive AML CD34+cells by the second-generation proteasome inhibitor carfilzomib

(2015) Lieke H. van der Helm et al.   BRITISH JOURNAL OF HAEMATOLOGY

Nanoparticle-based autoimmune disease therapy

(2015) Pau Serra et al.   CLINICAL IMMUNOLOGY

The dual nature of interleukin-10 in pemphigus vulgaris

(2015) Michael Jeffrey Cho et al.   CYTOKINE

Pemphigus Vulgaris: An Evidence-Based Treatment Update

(2015) Cathy Y. Zhao et al.   DRUGS

Distinctive Treg associated CCR4-CCL22 expression profile with altered frequency of Th17/Treg cell in the immunopathogenesis of Pemphigus Vulgaris

(2015) R. Asothai et al.   IMMUNOBIOLOGY

Chemotherapy-Refractory Diffuse Large B-Cell Lymphoma and Indolent B-Cell Malignancies Can Be Effectively Treated With Autologous T Cells Expressing an Anti-CD19 Chimeric Antigen Receptor

(2015) James N. Kochenderfer et al.   JOURNAL OF CLINICAL ONCOLOGY

Update on fogo selvagem, an endemic form of pemphigus foliaceus

(2015) Valeria Aoki et al.   JOURNAL OF DERMATOLOGY

Mucosal Pemphigus Vulgaris Anti-Dsg3 IgG Is Pathogenic to the Oral Mucosa of Humanized Dsg3 Mice

(2015) Donna A. Culton et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Persistence of Anti-Desmoglein 3 IgG + B-Cell Clones in Pemphigus Patients over Years

(2015) Christoph M. Hammers et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

T cells expressing CD19 chimeric antigen receptors for acute lymphoblastic leukaemia in children and young adults: a phase 1 dose-escalation trial

(2015) Daniel W Lee et al.   LANCET

Long-Term Remissions in Recalcitrant Pemphigus Vulgaris

(2015) A. Razzaque Ahmed et al.   NEW ENGLAND JOURNAL OF MEDICINE

Immune response in pemphigus and beyond: progresses and emerging concepts

(2015) Giovanni Di Zenzo et al.   Seminars in Immunopathology

Long-lived plasma cells are early and constantly generated in New Zealand Black/New Zealand White F1 mice and their therapeutic depletion requires a combined targeting of autoreactive plasma cells and their precursors

(2015) Adriano Taddeo et al.   ARTHRITIS RESEARCH & THERAPY

Evaluating drug-free remission with abatacept in early rheumatoid arthritis: results from the phase 3b, multicentre, randomised, active-controlled AVERT study of 24 months, with a 12-month, double-blind treatment period

(2014) Paul Emery et al.   ANNALS OF THE RHEUMATIC DISEASES

150thAnniversary Series: Desmosomes and Autoimmune Disease, Perspective of Dynamic Desmosome Remodeling and Its Impairments in Pemphigus

(2014) Yasuo Kitajima   CELL COMMUNICATION AND ADHESION

Systematic Review of Tofacitinib: A New Drug for the Management of Rheumatoid Arthritis

(2014) Kirandeep Kaur et al.   CLINICAL THERAPEUTICS

Molecular Diagnosis in Autoimmune Skin Blistering Conditions

(2014) J.V. Otten et al.   CURRENT MOLECULAR MEDICINE

Specific immunoadsorption of pathogenic autoantibodies in pemphigus requires the entire ectodomains of desmogleins

(2014) Jana Langenhan et al.   EXPERIMENTAL DERMATOLOGY

HLA DRB1* and DQB1* alleles are associated with disease severity in patients with pemphigus vulgaris

(2014) Danka Svecova et al.   INTERNATIONAL JOURNAL OF DERMATOLOGY

Novel System Evaluating In Vivo Pathogenicity of Desmoglein 3-Reactive T Cell Clones Using Murine Pemphigus Vulgaris

(2014) H. Takahashi et al.   JOURNAL OF IMMUNOLOGY

Involvement of the Apoptotic Mechanism in Pemphigus Foliaceus Autoimmune Injury of the Skin

(2014) N. Li et al.   JOURNAL OF IMMUNOLOGY

Pemphigus Vulgaris IgG Directly Inhibit Desmoglein 3-Mediated Transinteraction

(2014) W.-M. Heupel et al.   JOURNAL OF IMMUNOLOGY

A Single Helper T Cell Clone Is Sufficient to Commit Polyclonal Naive B Cells to Produce Pathogenic IgG in Experimental Pemphigus Vulgaris

(2014) H. Takahashi et al.   JOURNAL OF IMMUNOLOGY

Pathogenic IgG Antibodies against Desmoglein 3 in Pemphigus Vulgaris Are Regulated by HLA-DRB1*04:02–Restricted T Cells

(2014) Rüdiger Eming et al.   JOURNAL OF IMMUNOLOGY

No Evidence of Apoptotic Cells in Pemphigus Acantholysis

(2014) Ineke C. Janse et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Pemphigus. S2 Guideline for diagnosis and treatment - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)

(2014) M. Hertl et al.   JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY

Desmosomes and Extradesmosomal Adhesive Signaling Contacts in Pemphigus

(2014) Jens Waschke et al.   MEDICINAL RESEARCH REVIEWS

Alopecia areata is driven by cytotoxic T lymphocytes and is reversed by JAK inhibition

(2014) Luzhou Xing et al.   NATURE MEDICINE

Chimeric Antigen Receptor T Cells for Sustained Remissions in Leukemia

(2014) Shannon L. Maude et al.   NEW ENGLAND JOURNAL OF MEDICINE

Abatacept induces clinical improvement in patients with severe systemic sclerosis

(2014) FV de Paoli et al.   SCANDINAVIAN JOURNAL OF RHEUMATOLOGY

Shared VH1-46 gene usage by pemphigus vulgaris autoantibodies indicates common humoral immune responses among patients

(2014) Michael Jeffrey Cho et al.   Nature Communications

Subcutaneous Veltuzumab, a Humanized Anti-CD20 Antibody, in the Treatment of Refractory Pemphigus Vulgaris

(2014) Christoph T. Ellebrecht et al.   JAMA Dermatology

E-cadherin autoantibody profile in patients with pemphigus vulgaris

(2013) M.E.F. Oliveira et al.   BRITISH JOURNAL OF DERMATOLOGY

Autoreactive T cells in the immune pathogenesis of pemphigus vulgaris

(2013) Kyle T. Amber et al.   EXPERIMENTAL DERMATOLOGY

Peptide-mediated desmoglein 3 crosslinking prevents pemphigus vulgaris autoantibody-induced skin blistering

(2013) Volker Spindler et al.   JOURNAL OF CLINICAL INVESTIGATION

A higher correlation of the antibody activities against the calcium-dependent epitopes of desmoglein 3 quantified by ethylenediaminetetraacetic acid-treated enzyme-linked immunosorbent assay with clinical disease activities of pemphigus vulgaris

(2013) Koji Kamiya et al.   JOURNAL OF DERMATOLOGICAL SCIENCE

A New Light on an Old Disease: Adhesion Signaling in Pemphigus Vulgaris

(2013) Arnaud Galichet et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Pemphigus Vulgaris Autoantibody Profiling by Proteomic Technique

(2013) Mina Kalantari-Dehaghi et al.   PLoS One

Antigen-Specific Tolerance by Autologous Myelin Peptide-Coupled Cells: A Phase 1 Trial in Multiple Sclerosis

(2013) A. Lutterotti et al.   Science Translational Medicine

Long-Term Remissions of Severe Pemphigus After Rituximab Therapy Are Associated with Prolonged Failure of Desmoglein B Cell Response

(2013) N. Colliou et al.   Science Translational Medicine

Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study

(2012) Muriel Elhai et al.   ANNALS OF THE RHEUMATIC DISEASES

Detection of antibodies against the non-calcium-dependent epitopes of desmoglein 3 in pemphigus vulgaris and their pathogenic significance

(2012) K. Kamiya et al.   BRITISH JOURNAL OF DERMATOLOGY

Association between HLA-DRB1 polymorphisms and pemphigus vulgaris: a meta-analysis

(2012) L. Yan et al.   BRITISH JOURNAL OF DERMATOLOGY

Apoptotic Pathways in the Pathogenesis of Pemphigus: Targets for New Therapies

(2012) Roberta Lotti et al.   CURRENT PHARMACEUTICAL BIOTECHNOLOGY

Efficacy and safety of rituximab in pemphigus: experience of the German Registry of Autoimmune Diseases

(2012) Michael Kasperkiewicz et al.   JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT

Pathogenesis of NOD diabetes is initiated by reactivity to the insulin B chain 9-23 epitope and involves functional epitope spreading

(2012) Suchitra Prasad et al.   JOURNAL OF AUTOIMMUNITY

Pemphigus autoantibodies generated through somatic mutations target the desmoglein-3 cis-interface

(2012) Giovanni Di Zenzo et al.   JOURNAL OF CLINICAL INVESTIGATION

Cutting Edge: Brazilian Pemphigus Foliaceus Anti-Desmoglein 1 Autoantibodies Cross-React with Sand Fly Salivary LJM11 Antigen

(2012) Y. Qian et al.   JOURNAL OF IMMUNOLOGY

Differential IgG Recognition of Desmoglein 3 by Paraneoplastic Pemphigus and Pemphigus Vulgaris Sera

(2012) Oliver Brandt et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Epitope Spreading Is Rarely Found in Pemphigus Vulgaris by Large-Scale Longitudinal Study Using Desmoglein 2–Based Swapped Molecules

(2012) Bungo Ohyama et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Randomized double blind trial of prednisolone and azathioprine, vs. prednisolone and placebo, in the treatment of pemphigus vulgaris

(2012) C. Chams-Davatchi et al.   JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY

New insights into desmosome regulation and pemphigus blistering as a desmosome-remodeling disease

(2012) Yasuo Kitajima   KAOHSIUNG JOURNAL OF MEDICAL SCIENCES

Microparticles bearing encephalitogenic peptides induce T-cell tolerance and ameliorate experimental autoimmune encephalomyelitis

(2012) Daniel R Getts et al.   NATURE BIOTECHNOLOGY

Signaling Dependent and Independent Mechanisms in Pemphigus Vulgaris Blister Formation

(2012) Masataka Saito et al.   PLoS One

IgG Autoantibodies Against Desmocollin 3 in Pemphigus Sera Induce Loss of Keratinocyte Adhesion

(2011) David Rafei et al.   AMERICAN JOURNAL OF PATHOLOGY

The Extent of Desmoglein 3 Depletion in Pemphigus Vulgaris Is Dependent on Ca2+-Induced Differentiation

(2011) Volker Spindler et al.   AMERICAN JOURNAL OF PATHOLOGY

Beneficial effect of novel proteasome inhibitors in murine lupus via dual inhibition of type I interferon and autoantibody-secreting cells

(2011) H. Travis Ichikawa et al.   ARTHRITIS AND RHEUMATISM

Pemphigus autoimmunity: Hypotheses and realities

(2011) Sergei A. Grando   AUTOIMMUNITY

Antigen-independent development of Foxp3+ regulatory T cells suppressing autoantibody production in experimental pemphigus vulgaris

(2011) T. Yokoyama et al.   INTERNATIONAL IMMUNOLOGY

In vitro effects of perifosine, bortezomib and lenalidomide against hematopoietic progenitor cells from healthy donors

(2011) Martin Schmidt-Hieber et al.   INVESTIGATIONAL NEW DRUGS

Autoimmune bullous skin diseases. Part 2: diagnosis and therapy

(2011) Andrea Kneisel et al.   JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT

Autoimmune bullous skin diseases. Part 1: Clinical manifestations

(2011) Andrea Kneisel et al.   JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT

Generating Consensus Research Goals and Treatment Strategies for Pemphigus and Pemphigoid: The 2010 JC Bystryn Pemphigus and Pemphigoid Meeting

(2011) Heather Yarnall Schultz et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Desmoglein as a Target in Skin Disease and Beyond

(2011) Masayuki Amagai et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

An Adult Passive Transfer Mouse Model to Study Desmoglein 3 Signaling in Pemphigus Vulgaris

(2011) Katja Schulze et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Regulatory T-Cell Responses to Low-Dose Interleukin-2 in HCV-Induced Vasculitis

(2011) David Saadoun et al.   NEW ENGLAND JOURNAL OF MEDICINE

Interleukin-2 and Regulatory T Cells in Graft-versus-Host Disease

(2011) John Koreth et al.   NEW ENGLAND JOURNAL OF MEDICINE

Autoimmunity to Desmocollin 3 in Pemphigus Vulgaris

(2010) Xuming Mao et al.   AMERICAN JOURNAL OF PATHOLOGY

The efficacy and safety of abatacept in patients with non-life-threatening manifestations of systemic lupus erythematosus: Results of a twelve-month, multicenter, exploratory, phase IIb, randomized, double-blind, placebo-controlled trial

(2010) J. T. Merrill et al.   ARTHRITIS AND RHEUMATISM

Binding of pemphigus vulgaris IgG to antigens in desmosome core domains excludes immune complexes rather than directly splitting desmosomes

(2010) Y. Aoyama et al.   BRITISH JOURNAL OF DERMATOLOGY

Immune response towards the amino-terminus of desmoglein 1 prevails across different activity stages in nonendemic pemphigus foliaceus

(2010) P.T. Chan et al.   BRITISH JOURNAL OF DERMATOLOGY

Novel ELISA systems for antibodies to desmoglein 1 and 3: correlation of disease activity with serum autoantibody levels in individual pemphigus patients

(2010) Enno Schmidt et al.   EXPERIMENTAL DERMATOLOGY

Treating Pemphigus Vulgaris with Prednisone and Mycophenolate Mofetil: A Multicenter, Randomized, Placebo-Controlled Trial

(2010) Stefan Beissert et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Desmosome Disassembly in Response to Pemphigus Vulgaris IgG Occurs in Distinct Phases and Can Be Reversed by Expression of Exogenous Dsg3

(2010) Jean M. Jennings et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

The Protease Inhibitor Alpha-2-Macroglobuline-Like-1 Is the p170 Antigen Recognized by Paraneoplastic Pemphigus Autoantibodies in Human

(2010) Isabelle Schepens et al.   PLoS One

ELISA Testing of Anti–Desmoglein 1 and 3 Antibodies in the Management of Pemphigus

(2009) Claire Abasq et al.   ARCHIVES OF DERMATOLOGY

Detection of Autoantibodies against Recombinant Desmoglein 1 and 3 Molecules in Patients with Pemphigus vulgaris: Correlation with Disease Extent at the Time of Diagnosis and during Follow-Up

(2009) Anna Belloni-Fortina et al.   Clinical & Developmental Immunology

Clinical activity of pemphigus vulgaris relates to IgE autoantibodies against desmoglein 3

(2009) Angela Nagel et al.   CLINICAL IMMUNOLOGY

Induction of hyper-adhesion attenuates autoimmune-induced keratinocyte cell–cell detachment and processing of adhesion molecules via mechanisms that involve PKC

(2009) Nicola Cirillo et al.   EXPERIMENTAL CELL RESEARCH

Involvement of TH1/TH2 Cytokines in the Pathogenesis of Autoimmune Skin Disease—Pemphigus Vulgaris

(2009) Abhigyan Satyam et al.   IMMUNOLOGICAL INVESTIGATIONS

Biphasic Activation of p38MAPK Suggests That Apoptosis Is a Downstream Event in Pemphigus Acantholysis

(2009) Hua En Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Antimitochondrial Autoantibodies in Pemphigus Vulgaris

(2009) Steve Marchenko et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Antibodies to the Desmoglein 1 Precursor Proprotein but Not to the Mature Cell Surface Protein Cloned from Individuals without Pemphigus

(2009) J. Yamagami et al.   JOURNAL OF IMMUNOLOGY

Pathogenic Anti-Desmoglein MAbs Show Variable ELISA Activity because of Preferential Binding of Mature versus Proprotein Isoforms of Desmoglein 3

(2009) Preety M. Sharma et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

The final destiny of acantholytic cells in pemphigus is Fas mediated

(2009) MG Pacheco-Tovar et al.   JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY

The as-yet unfulfilled promise of p38 MAPK inhibitors

(2009) Susan E. Sweeney   Nature Reviews Rheumatology

HLA Class II (DRB, DQA1 and DQB1) Allele and Haplotype Frequencies in the Patients with Pemphigus Vulgaris

(2008) Sheida Shams et al.   JOURNAL OF CLINICAL IMMUNOLOGY

E-cadherin Is an Additional Immunological Target for Pemphigus Autoantibodies

(2008) Flor Evangelista et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Rituximab Exerts a Dual Effect in Pemphigus Vulgaris

(2008) Rüdiger Eming et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Serum Concentration of Interleukin-6 Is Increased Both in Active and Remission Stages of Pemphigus Vulgaris

(2008) Joanna Narbutt et al.   MEDIATORS OF INFLAMMATION

Isolation of Pathogenic Monoclonal Anti-Desmoglein 1 Human Antibodies by Phage Display of Pemphigus Foliaceus Autoantibodies

(2007) Ken Ishii et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Induction of p38MAPK and HSP27 Phosphorylation in Pemphigus Patient Skin

(2007) Paula Berkowitz et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY