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    High-dose intravenous immunoglobulins for the treatment of dermatological autoimmune diseases.
    J Dtsch Dermatol Ges 2017 Dec;15(12):1211-1226
    Department of Dermatology, University of Heidelberg, Heidelberg, Germany.
    Based on their immunomodulatory properties, high-dose intravenous immunoglobulins (IVIGs) are successfully used in the treatment of various dermatological autoimmune diseases, in particular pemphigus vulgaris and dermatomyositis. In autoimmune bullous diseases, IVIGs can be used in an adjuvant setting (second- or third-line therapy) once combined immunosuppressive regimens have failed. In dermatomyositis, IVIGs may already be employed as an adjuvant second-line therapy after failure of corticosteroid monotherapy. Read More

    A Review of the Effectiveness and Side-Effects of Fluocinolone Acetonide 0.1% in the Treatment of Oral Mucosal Diseases.
    Acta Stomatol Croat 2017 Sep;51(3):240-247
    Oral Medicine Department, Faculty of Dentistry Chulalongkorn University Bangkok, Thailand.
    Topical steroids have been widely used in the treatment of symptomatic oral lesions to reduce pain and inflammation. Potent topical steroids such as clobetasol propionate, fluocinolone acetonide (FA), and fluocinonide have been widely used in the treatment of severe oral mucosal lesions. Many reports have demonstrated that these steroids were effective in treating oral lesions with only minor side-effects. Read More

    Cell junction protein armadillo repeat gene deleted in velo-cardio-facial syndrome is expressed in the skin and colocalizes with autoantibodies of patients affected by a new variant of endemic pemphigus foliaceus in Colombia.
    Dermatol Pract Concept 2017 Oct 31;7(4):3-8. Epub 2017 Oct 31.
    Georgia Dermatopathology Associates, Atlanta, GA, USA.
    Background: We previously described a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America (El Bagre-EPF, or pemphigus Abreu-Manu). El Bagre-EPF differs from other types of EPF clinically, epidemiologically, immunologically and in its target antigens. We reported the presence of patient autoantibodies colocalizing with armadillo repeat gene deleted in velo-cardio-facial syndrome (ARVCF), a catenin cell junction protein colocalizing with El Bagre-EPF autoantibodies in the heart and within pilosebaceous units along their neurovascular supply routes. Read More

    Oral Manifestation in Patients diagnosed with Dermatological Diseases.
    J Contemp Dent Pract 2017 Dec 1;18(12):1153-1158. Epub 2017 Dec 1.
    Department of Oral Pathology and Microbiology, M. A. Rangoonwala College of Dental Sciences & Research Centre Pune, Maharashtra, India.
    Aim: This study aimed to estimate the frequency of oral lesions in dermatological diseased patients attending the outpatient department of Pravara Rural Medical and Dental College and Hospital, Pravara Institute of Medical Sciences (Deemed University), Loni, Ahmednagar, Maharashtra, India, and the Department of Dermatology at Medical College, Ahmednagar, Maharashtra, India.

    Materials And Methods: A cross-sectional hospital-based study was conducted in Loni from October 2013 to January 2014. A total of 310 patients (mean age 37. Read More

    Paraneoplastic Pemphigus with Underlying Retroperitoneal Inflammatory Myofibroblastic Tumor: A Case Report and Review of the Literature.
    Indian Dermatol Online J 2017 Nov-Dec;8(6):478-481
    Department of Pharmacology, Islamic Azad University, Science and Research Branch, Tehran, Iran.
    Inflammatory myofibroblastic tumor (IMT) is a peculiar low-grade neoplasm of spindle cell fibroblasts and myofibroblasts in an inflammatory background. The lung is the most common site of involvement. Here, we report a case of paraneoplastic pemphigus (PNP)-associated with an extensive retroperitoneal IMT. Read More

    Cytokine Indexes in Pemphigus Vulgaris: Perception of Its Immunpathogenesis and Hopes for Non-Steroidal Treatment.
    Iran J Pharm Res 2017 ;16(3):1223-1229
    Department of Dermatology, Isfahan University of Medical Science, Isfahan, Iran.
    Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease of the skin, in which loss of adhesion between keratinocytes is caused by autoantibodies. It has been hypothesized that cytokines play an essential role in the pathogenesis of PV. This study aimed to investigate the other immunopathological aspects of PV by determining the serum levels of cytokines in PV patients to find another treatment strategy except corticosteroid therapy. Read More

    Pemphigus and hematologic malignancies: A population-based study of 11,859 patients.
    J Am Acad Dermatol 2017 Nov 30. Epub 2017 Nov 30.
    Department of Quality Measurements and Research, Chief Physician's Office, Clalit Health Services, Tel Aviv, Israel; Siaal Research Center for Family Medicine and Primary Care, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.
    Background: The association of non-paraneoplastic pemphigus with comorbid hematologic malignancies is yet to be established.

    Objective: To estimate the association between pemphigus and the common types of hematologic malignancies.

    Methods: A cross-sectional study was conducted comparing pemphigus patients with age-, sex- and ethnicity-matched control subjects regarding the prevalence of 6 comorbid hematologic malignancies. Read More

    Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy.
    Int J Mol Sci 2017 Nov 26;18(12). Epub 2017 Nov 26.
    Dipartimento di Medicina Interna e Specialità Mediche, Dermatologia, Sapienza Università di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy.
    Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Read More

    Association between HLA-DQB1 polymorphisms and pemphigus vulgaris: A meta-analysis.
    Immunol Invest 2017 Nov 28:1-12. Epub 2017 Nov 28.
    a Department of Epidemiology and Biostatistics , School of Public Health, Anhui Medical University , Hefei , Anhui , China.
    Objective: This study was performed to systematically summarize the results on the association of HLA-DQB1 polymorphisms with pemphigus vulgaris (PV) and other related factors.

    Methods: A comprehensive literature search of PubMed, The Cochrane Library, Embase, and Google Scholar database was conducted to identify relevant articles in English, with the last report up to November 1, 2016. Heterogeneity test was performed, and publication bias was evaluated. Read More

    Evaluation of C-reactive protein as an inflammatory marker of pemphigus foliaceus and superficial pyoderma in dogs.
    Vet Dermatol 2017 Nov 22. Epub 2017 Nov 22.
    Department of Clinical Science, School of Veterinary Medicine, University of São Paulo, Av. Prof. Orlando M. Paiva, 87, São Paulo, SP, 05508-270, Brazil.
    Background: C-reactive protein (CRP) is a major acute phase protein in dogs and may be an inflammatory marker of autoimmune diseases.

    Hypothesis/objectives: To measure pre- and post-treatment CRP levels in dogs with superficial pyoderma (SP) and pemphigus foliaceus (PF) compared to normal dogs; to evaluate possible correlation between CRP values, indirect immunofluorescence (IIF), and pemphigus foliaceus extent and severity index (PEFESI) scores.

    Animals: Fifty nine dogs divided into three groups: Group I (control, n = 31), Group II (SP, n = 14) and Group III (PF, n = 14). Read More


    New insights into pemphigoid diseases.
    Exp Dermatol 2017 Nov 21. Epub 2017 Nov 21.
    Lübeck Institute of Experimental Dermatology, University of Lübeck, Germany.
    Pemphigus and pemphigoid diseases are organ-specific autoimmune blistering diseases (AIBD), characterized and caused by autoantibodies to structural components of the skin (1). The autoantigens targeted in pemphigus are desmoglein 1 and 3, two proteins of the desmosomal structure, while the autoantigens in pemphigoid diseases (PD) are components of the basal membrane. For example, bullous pemphigoid (BP), the most frequent PD is characterized by autoantibodies against type XVII collagen (COL17, BP180) and BP230, and epidermolysis bullosa acquisita (EBA) is caused by autoantibodies against type VII collagen (COL7). Read More

    Diagnosis of Castleman Disease.
    Hematol Oncol Clin North Am 2018 Feb;32(1):53-64
    Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, M230 Boston, MA 02215, USA; VA Boston Healthcare System, 1400 VFW Parkway, West Roxbury, MA, USA. Electronic address:
    Castleman disease (CD) is a rare and heterogenous group of disorders sharing in common an abnormal lymph node pathology. CD comprises distinct subtypes with different prognoses. Unicentric CD and multicentric CD are featured by specific systemic manifestations and may be associated with Kaposi sarcoma, non-Hodgkin and Hodgkin lymphoma, and POEMS syndrome. Read More

    Autoantibodies to full body vascular cell junctions colocalize with MYZAP, ARVCF, desmoplakins I and II and p0071 in endemic pemphigus in Colombia, South America.
    Int J Dermatol 2017 Nov 20. Epub 2017 Nov 20.
    Georgia Dermatopathology Associates, Atlanta, GA, USA.
    Background: We previously described a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF).

    Methods: Here we aimed to investigate disease autoreactivity to vessels in all body organs/systems. We compared 57 patients and 57 controls from the endemic area, matched by demographics, age, sex, and work activity. Read More

    Evaluation of anti-desmoglein-1 and anti-desmoglein-3 autoantibody titers in pemphigus patients at the time of the initial diagnosis and after clinical remission.
    Medicine (Baltimore) 2017 Nov;96(46):e8801
    Unit of Dermatology, University of Padua, Padova, Italy.
    It has been suggested that anti-desmoglein autoantibody titers could be helpful in follow-up and therapeutic management of pemphigus patients. However, there is no consensus regarding the relationship between anti-desmoglein autoantibody titers and clinical activity of pemphigus.The aim of our study was to evaluate if clinical remission of pemphigus relates to the presence of anti-desmoglein autoantibodies. Read More

    Association between pemphigus and psoriasis: A population-based large-scale study.
    J Am Acad Dermatol 2017 Dec;77(6):1174-1175
    Department of Quality Measurements and Research, Chief Physician's Office, Clalit Health Services, Tel Aviv, Israel; Siaal Research Center for Family Medicine and Primary Care, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

    Sixteen-year history of rituximab therapy for 1085 pemphigus vulgaris patients: A systematic review.
    Int Immunopharmacol 2017 Nov 10;54:131-138. Epub 2017 Nov 10.
    Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    Pemphigus vulgaris (PV) is a rare autoimmune disease due to the production of pathogenic autoantibodies directed against desmoglein 1 and 3, usually affecting both skin and mucous membranes. Recently, rituximab, a chimeric IgG1 monoclonal antibody which targets the CD20 molecules have been regarded as a promising treatment for PV. In this study, a systematic review was conducted to conclude on how and which PV patients could benefit from rituximab infusion. Read More

    Bullous Diseases.
    Curr Probl Dermatol 2018 7;53:64-69. Epub 2017 Nov 7.
    Autoimmune bullous diseases are a heterogeneous group of blistering diseases affecting the skin and/or mucous membrane. Systemic corticosteroids, which are often associated with immunosuppressants, are the main treatment option for these diseases. The 2 main biologics used in the treatment of autoimmune bullous diseases are rituximab, especially in pemphigus and mucous membrane pemphigoid, and omalizumab in bullous pemphigoid. Read More

    Antidesmoglein 1 and 3 antibodies in healthy subjects of a population in the Peruvian high amazon.
    Int J Dermatol 2017 Nov 11. Epub 2017 Nov 11.
    Department of Dermatology, Oregon Health & Science University, Portland, OR, USA.
    Background: The objective of this study was to determine the presence of anti-Dsg1 and Dsg3 antibodies in healthy subjects of the high Peruvian Amazon (Tuemal, Rodriguez de Mendoza province, department of Amazonas) to establish the theoretical presence of environmental factors or triggers in the area.

    Materials And Methods: Cross-sectional study. The study population included persons of any age or gender, clinically healthy, who were evaluated by a dermatologist to confirm the absence of blistering diseases. Read More

    Acute Flare of Bullous Pemphigus With Pembrolizumab Used for Treatment of Metastatic Urothelial Cancer.
    J Immunother 2017 Nov 3. Epub 2017 Nov 3.
    Departments of *Hematology and Oncology †Internal Medicine ‡Dermatology, University of Iowa Hospitals and Clinics, Iowa City, IA.
    In the past decade, the resurgence of immunotherapy has changed the landscape of cancer therapy. Checkpoint inhibitors targeting cytotoxic T-lymphocyte antigen-4, programmed death-1 on lymphocytes, and programmed death ligand-1 on tumors cells are currently utilized in the management of several cancers. These agents are double-edged sword with the positive effect being robust antitumor response but on the other side they can throttle up the normal immunologic homeostasis in a negative way, leading to adverse autoimmune toxicities. Read More

    Pemphigus trigger factors: special focus on pemphigus vulgaris and pemphigus foliaceus.
    Arch Dermatol Res 2017 Nov 6. Epub 2017 Nov 6.
    Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    Pemphigus is a general term for a rare group of autoimmune diseases which result in the formation of blisters on the skin and oral cavity. Although there is no way to prevent autoimmune diseases, some factors may trigger pemphigus initiation in susceptible individuals or be exacerbated in affected patients. Recognition of these triggers, based on the latest studies and experiences is essential and should be updated every few years. Read More

    Bipolar Disorder Associated with Another Autoimmune Disease-Pemphigus: A Population-based Study.
    Can J Psychiatry 2017 Jan 1:706743717740344. Epub 2017 Jan 1.
    3 Department of Quality Measurements and Research, Chief Physician's Office, Clalit Health Services, Tel Aviv, Israel.
    Objectives: Recent evidence suggests a notable role for inflammation and immune dysregulation in the neuroprogression of bipolar disorders (BD). Several autoimmune comorbidities have been reported in association with BD. However, the epidemiological relationship between pemphigus and BD has not yet been elucidated. Read More

    Current and future treatment options for pemphigus: Is it time to move towards more effective treatments?
    Int Immunopharmacol 2017 Dec;53:133-142
    Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Electronic address:
    Systemic administration of corticosteroid (CS) remains the standard gold treatment for pemphigus. However, because of several long-term adverse effects, steroid-sparing agents are usually prescribed in combination with CSs. Despite the high number of available studies, the choice of best drugs to treat pemphigus remains controversial. Read More

    Successful and well-tolerated bi-weekly immunoadsorption regimen in pemphigus vulgaris.
    Atheroscler Suppl 2017 Nov 3;30:271-277. Epub 2017 Jun 3.
    Department of Dermatology, University Hospital and Carl Gustav Carus Faculty of Medicine, Technische Universität Dresden, Dresden, Germany. Electronic address:
    Background: Pemphigus vulgaris is a chronic autoimmune disease characterized by blisters and erosions forming in the mucous membranes and the skin. Many patients are severely impaired by pain, weight loss and increased risk of infections. The disease is mediated by specific autoantibodies directed against desmogleins that contribute to connect keratinocytes in the epidermis. Read More

    Anterior Scleritis Associated with Pemphigus Vulgaris.
    Ocul Immunol Inflamm 2017 Nov 2:1-2. Epub 2017 Nov 2.
    a Department of Dermatology , Tel Aviv Sourasky Medical Centre , Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv , Israel.
    We present a rare co-occurrence of anterior scleritis developing in a patient with recalcitrant pemphigus vulgaris. We discuss the clinical and therapeutic implications of these two conditions and offer concise recommendations. Read More

    Grover's disease: dermoscopy, reflectance confocal microscopy and histopathological correlation.
    Dermatol Pract Concept 2017 Jul 31;7(3):51-54. Epub 2017 Jul 31.
    Dermatology Clinic, University of Catania, Italy.
    Grover's disease is a benign condition of unknown origin characterized clinically by an erythematous papulovesicular eruption and histopathologically by intraepidermal clefting and four different patterns of acantholysis: Darier-like, pemphigus-like, spongiotic, and Hailey-Hailey-like. A case of a 54-year-old female affected by Grover's disease and showing a Darier-like histopathological pattern is described. Polarized light dermoscopy (PLD) revealed the presence of polygonal, star-like shaped yellowish/brownish areas of various sizes surrounded by a thin whitish halo. Read More

    The Effect of Low Level Laser Therapy on Pemphigus Vulgaris Lesions: A Pilot Study.
    J Lasers Med Sci 2017 27;8(4):177-180. Epub 2017 Sep 27.
    Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    Introduction: Pemphigus vulgaris is a chronic blistering skin disease. Management of recalcitrant pemphigus ulcers is a great problem. Low Level Laser Therapy (LLLT) is known to supply direct biostimulative light energy to body cells. Read More

    Antioxidant status of serum bilirubin, uric acid and albumin in pemphigus vulgaris.
    Clin Exp Dermatol 2017 Oct 25. Epub 2017 Oct 25.
    Department of Clinical Laboratory, First Affiliated Hospital of Guangxi Medical University, Nanning, China.
    Background: Oxidative stress and low antioxidant status are implicated in the pathogenesis of inflammatory and autoimmune diseases. Pemphigus vulgaris (PV) is an extremely severe autoimmune bullous dermatosis characterized by intraepithelial bullae on the skin and mucosa, and its antioxidant status is not fully understood.

    Aim: To assess correlations between PV and serum antioxidant levels of bilirubin, uric acid (UA) and albumin. Read More

    Peripheral CD19hi B cells exhibit activated phenotype and functionality in promoting IgG and IgM production in human autoimmune diseases.
    Sci Rep 2017 Oct 24;7(1):13921. Epub 2017 Oct 24.
    Shanghai Institute of Immunology, Shanghai Jiao Tong University School of Medicine, 200025, Shanghai, P. R. China.
    Systemic Lupus Erythematosus (SLE) and pemphigus are two representative autoimmune diseases driven by pathogenic autoantibody systemically and organ-specifically, respectively. Given the involvement of antibody in the pathogenesis, B cells are inclined to differentiate and function in an abnormal activation model. Here we defined a unique CD19hi B cell population existing in the periphery of SLE and pemphigus patients as well as in human tonsils. Read More

    Rituximab in pemphigus.
    Immunotherapy 2018 Jan 24;10(1):27-37. Epub 2017 Oct 24.
    Department of Dermatology, Rouen University Hospital, & INSERM U 1234, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France.
    Pemphigus is a severe autoimmune blistering disease mediated by pathogenic anti-desmoglein antibodies leading to an inter keratinocyte disjunction. Rituximab is a monoclonal antibody that binds to the CD-20 antigen of B lymphocytes, which causes B-cell depletion and a subsequent reduction in pathogenic autoantibodies. Its therapeutic role in pemphigus has been progressively growing with increasing evidence of successful outcomes. Read More

    Desmoglein 3-Reactive B Cells "Hiding" in Pemphigus Lesions.
    J Invest Dermatol 2017 Nov;137(11):2255-2257
    Department of Dermatology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan. Electronic address:
    Pemphigus vulgaris is an autoimmune blistering disease caused by anti-desmoglein 3 IgG autoantibodies. It is accepted that interactions between autoreactive B and T cells are key to humoral autoimmunity targeting desmoglein 3. This orchestrated process usually occurs in secondary lymphoid organs, including the spleen and lymph nodes. Read More

    Human papillomavirus vaccination of adult women and risk of autoimmune and neurological diseases.
    J Intern Med 2017 Oct 18. Epub 2017 Oct 18.
    Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden.
    Background: Since 2006, human papillomavirus (HPV) vaccines have been introduced in many countries worldwide. Whilst safety studies have been reassuring, focus has been on the primary target group, the young adolescent girls. However, it is also important to evaluate safety in adult women where background disease rates and safety issues could differ significantly. Read More

    Mechanisms Causing Loss of Keratinocyte Cohesion in Pemphigus.
    J Invest Dermatol 2017 Oct 13. Epub 2017 Oct 13.
    Institute of Anatomy and Cell Biology, Ludwig-Maximilians-Universität, Munich, Germany. Electronic address:
    The autoimmune blistering skin disease pemphigus is caused by IgG autoantibodies against desmosomal cadherins, but the precise mechanisms are in part a matter of controversial discussions. This review focuses on the currently existing models of the disease and highlights the relevance of desmoglein-specific versus nondesmoglein autoantibodies, the contribution of nonautoantibody factors, and the mechanisms leading to cell dissociation and blister formation in response to autoantibody binding. As the review brings together the majority of laboratories currently working on pemphigus pathogenesis, it aims to serve as a solid basis for further investigations for the entire field. Read More

    Urocytological evaluation of pemphigus patients on long term cyclophosphamide therapy: A cross sectional study.
    Indian J Dermatol Venereol Leprol 2017 Nov-Dec;83(6):667-672
    Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.
    Background: Cyclophosphamide therapy is associated with several urological complications including urinary bladder malignancy. Data on urologic complications of chronic cyclophosphamide therapy for dermatologic conditions is not available.

    Objectives: To study the urocytological profile of pemphigus patients on long-term cyclophosphamide therapy. Read More

    Patients with a new variant of endemic pemphigus foliaceus have autoantibodies against arrector pili muscle, colocalizing with MYZAP, p0071, desmoplakins 1 and 2 and ARVCF.
    Clin Exp Dermatol 2017 Dec 15;42(8):874-880. Epub 2017 Oct 15.
    Georgia Dermatopathology Associates, Atlanta, GA, USA.
    Background: We identified a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America, which we term El Bagre-EPF, and observed reactivity to arrector pili muscle (APM), thus we tested for autoimmunity to APM.

    Methods: We took skin biopsies from 30 patients with El Bagre-EPF and 30 healthy controls (HCs) matched by age, sex and occupation, who were all from the endemic area, and tested these using direct immunofluorescence (DIF), confocal microscopy, immunohistochemistry and immunoblotting (IB).

    Results: Of the 30 patients with El Bagre-EPF, 27 had autoantibodies to APM that colocalized with commercial antibodies to myocardium-enriched zonula occludens-1-associated protein (MYZAP), desmoplakin (DP)1 and DP2, plakophilin 4, and Armadillo repeat gene deleted in velo-cardio-facial syndrome (ARVCF) (P < 0. Read More

    Spectrum of orocutaneous disease associations: Immune-mediated conditions.
    J Am Acad Dermatol 2017 Nov;77(5):795-806
    Division of Dermatology, Baylor University Medical Center, Dallas, Texas. Electronic address:
    There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies. Read More

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