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    Anti-Thyroid Peroxidase Reactivity Is Heightened in Pemphigus Vulgaris and Is Driven by Human Leukocyte Antigen Status and the Absence of Desmoglein Reactivity.
    Front Immunol 2018 5;9:625. Epub 2018 Apr 5.
    Department of Dermatology, Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, United States.
    Pemphigus vulgaris (PV) belongs to an autoimmune disease cluster that includes autoimmune thyroid disease (AITD), suggesting common mechanisms driving autoimmune susceptibility. Our group has shown that PV patients exhibit significant reactivity to AITD-related anti-thyroid peroxidase (anti-TPO), and anti-TPO antibodies affect signaling pathways in keratinocytes similar to anti-desmoglein (Dsg) 3 antibodies. To further assess the relevance of anti-TPO reactivity in PV, we analyzed anti-TPO levels in 280 PV and 167 healthy control serum samples across a comprehensive set of variable and static parameters of disease activity and etiopathogenesis. Read More

    Electrocardiographic changes after high dose corticosteroid pulse therapy in pemphigus patients.
    J Dermatolog Treat 2018 Apr 18:1-14. Epub 2018 Apr 18.
    d The Skin Clinic , Williamstown , Massachusetts , USA.
    Purpose: The objective of the current study was to evaluate the effect of intravenous methylprednisolone pulse therapy on myocardial repolarization alterations, especially QT dispersion in anticipation of cardiovascular risks in patients with pemphigus vulgaris.

    Methods: The current study is a prospective controlled study on the electrocardiographic changes after methylprednisolone pulse therapy in 50 patients with pemphigus vulgaris.

    Results: A total of 50 patients (28 female and 22 male) were included in our study. Read More

    Atomic Force Microscopy Provides New Mechanistic Insights into the Pathogenesis of Pemphigus.
    Front Immunol 2018 28;9:485. Epub 2018 Mar 28.
    Institute of Anatomy, Faculty of Medicine, Ludwig-Maximilians-Universität München, Munich, Germany.
    Autoantibodies binding to the extracellular domains of desmoglein (Dsg) 3 and 1 are critical in the pathogenesis of pemphigus by mechanisms leading to impaired function of desmosomes and blister formation in the epidermis and mucous membranes. Desmosomes are highly organized protein complexes which provide strong intercellular adhesion. Desmosomal cadherins such as Dsgs, proteins of the cadherin superfamily which interact their extracellular domains in Ca-dependent manner, are the transmembrane adhesion molecules clustered within desmosomes. Read More

    Chronic oral lichenoid erosions revealing haematological malignancies.
    J Eur Acad Dermatol Venereol 2018 Apr 6. Epub 2018 Apr 6.
    Dermatology Department, Saint-Louis Hospital, Paris, France.
    Lichen planus is a chronic inflammatory disease of the skin and oral mucosa involving CD8+ T cells (1). Paraneoplastic pemphigus (PNP) is an autoimmune blistering skin disease associated with solid cancer or haematological malignancies (HM). We report 4 patients with long term oral lichenoid erosions leading to the diagnosis of PNP associated with HM. Read More

    Paraneoplastic pemphigus without antibodies to desmoglein 1 and 3.
    Dermatol Online J 2018 Feb 15;24(2). Epub 2018 Feb 15.
    Feinberg School of Medicine, Northwestern University, Chicago, Illinois,.
    Paraneoplastic pemphigus is a severe autoimmune blistering disease presenting in the setting of underlying malignancy. Paraneoplastic pemphigus is associated with diffuse painful stomatitis throughout the oral cavity with extension to the lips. The cutaneous findings are varied and have been described as lichenoid, pemphigoid, and targetoid lesions. Read More

    Th1/Th17 cell recognition of desmoglein 3 and bullous pemphigoid antigen 180 in lichen planus.
    J Allergy Clin Immunol 2018 Apr 4. Epub 2018 Apr 4.
    Department of Dermatology and Allergology, Philipps-University, Marburg, Germany. Electronic address:
    We identified Th1/Th17 cell responses against desmoglein 3 and bullous pemphigoid antigen 180 in lichen planus. In contrast, patients with pemphigus vulgaris and bullous pemphigoid showed significantly higher Th2 cell responses against these autoantigens. Read More

    Keratins Regulate p38MAPK-Dependent Desmoglein Binding Properties in Pemphigus.
    Front Immunol 2018 19;9:528. Epub 2018 Mar 19.
    Faculty of Medicine, Institute of Anatomy, Ludwig-Maximilians-Universität München, Munich, Germany.
    Keratins are crucial for the anchorage of desmosomes. Severe alterations of keratin organization and detachment of filaments from the desmosomal plaque occur in the autoimmune dermatoses pemphigus vulgaris and pemphigus foliaceus (PF), which are mainly caused by autoantibodies against desmoglein (Dsg) 1 and 3. Keratin alterations are a structural hallmark in pemphigus pathogenesis and correlate with loss of intercellular adhesion. Read More

    Psoriasiform Pemphigus Foliaceus in an African American Female: An Important Clinical Manifestation.
    J Drugs Dermatol 2018 Apr;17(4):471-473
    A 50-year-old African-American woman presented to the dermatology clinic with a pruritic eruption of 3 years' duration. On clinical examination, the patient had well-demarcated, pink, atrophic plaques and superficial erosions over the inframammary folds and mid-chest. She also had well-demarcated, hyperpigmented, hyperkeratotic scaly plaques over the abdomen, suprapubic region, elbows, knees, and back with sporadic small superficial blisters. Read More

    Association between human leukocyte antigen-DRB1 and human leukocyte antigen-DQB1 alleles and pemphigus vulgaris in Indian patients: A case-control study.
    Indian J Dermatol Venereol Leprol 2018 May-Jun;84(3):280-284
    Department of Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India.
    Background: HLA-DRB1*04, -DRB1*08, -DRB1*14, -DQB1*03 and -DQB1*05 are reported to have significant association with pemphigus vulgaris; however, this is partially dependent on ethnicity. This study was done to determine the HLA-DR and -DQ types prevalent in Indian patients with pemphigus vulgaris.

    Methods: A prospective case-control study was done for a period of 9 months in Christian Medical College Vellore, India. Read More

    Regulatory T cells in autoimmune skin diseases.
    Exp Dermatol 2018 Mar 25. Epub 2018 Mar 25.
    Department of Dermatology, Hokkaido University Graduate School of Medicine.
    CD4 Foxp3 regulatory T cells (Tregs) are suppressors of immune activation and play a crucial role in the maintenance of peripheral tolerance. Mutations of Foxp3 result in fatal autoimmunity in multiple organs, including the skin, in both humans and mice. Many studies have demonstrated the altered frequency and functions of Tregs, changes in cytokine and chemokine levels related to Tregs, and the differences in genetic background regarding Tregs in autoimmune skin disorders. Read More

    Association of serious infections with pemphigus and pemphigoid: analysis of the Nationwide Inpatient Sample.
    J Eur Acad Dermatol Venereol 2018 Mar 25. Epub 2018 Mar 25.
    Departments of Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
    Background: Pemphigus and pemphigoid are blistering disorders associated with barrier disruption, immune dysregulation and use of immunosuppressing systemic therapy, which may predispose toward serious infections.

    Objectives: To determine whether pemphigus and pemphigoid are associated with increased likelihood of serious infections, and the impact of such infections on mortality and cost of care.

    Methods: We analyzed data from the 2002-2012 National Inpatient Sample, including a representative 20% sample of all hospitalizations in the US (total n=72,108,077 adults). Read More

    American council on ECP (ACE): Why now?
    J Clin Apher 2018 Mar 25. Epub 2018 Mar 25.
    Department of Pediatric Hematology/Oncology/Neuro-oncology/Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University, Chicago, Illinois.
    Stimulated by the scientific progress in deciphering the principal elements contributing to the clinical efficacy of extracorporeal photochemotherapy (ECP), the American Council on ECP (ACE) was formed, under the auspices of the American Society for Apheresis (ASFA), to develop a field-guiding Consensus Report. ACE is composed of thirty nationally recognized ECP experts, clinically spanning cancer, transplantation, and autoimmunity and scientifically bridging immunology, bioengineering, and hematology. The two-day meeting took place in Manhattan, April 13-14, 2017, and unanimous consensus on nine pivotal points is herein reported. Read More

    Herpetiform Pemphigus with Characteristic Transmission Electron Microscopic Findings of Various-sized Ballooning Vacuoles in Keratinocytes without Acantholysis.
    Br J Dermatol 2018 Mar 24. Epub 2018 Mar 24.
    Department of Dermatology, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku, Tokyo, 162-8655, Japan.
    We report a unique Japanese female case of herpetiform pemphigus (HP) who had immunoglobulin G (IgG) autoantibodies reactive with non-desmosomal sites of keratinocytes, and presented characteristic transmission electron microscopic (TEM) findings of various-sized vacuoles in keratinocytes without acantholysis. The patient presented with pruritic annular edematous erythemas with small blisters lining at the margins on the trunk and extremities. Histopathological examinations showed intraepidermal blisters with prominent infiltrations of eosinophils. Read More

    Commentary on "Changing prevalence of diabetes mellitus in bullous pemphigoid: It is the dipeptidyl peptidase-4 inhibitors".
    J Eur Acad Dermatol Venereol 2018 Mar 23. Epub 2018 Mar 23.
    Istituto Dermopatico dell'Immacolata-IRCCS, FLMM, via dei Monti di Creta 104, 00167, Rome, Italy.
    In their article entitled "Changing prevalence of diabetes mellitus in bullous pemphigoid: It is the dipeptidyl peptidase-4 inhibitors" Gravani and colleagues confirmed the increasing incidence of bullous pemphigoid (BP) in the last decade, and highlighted its association with diabetes mellitus (DM), and specifically with dipeptidyl peptidase-4 inhibitors (DPP-4-i). As the Authors noted, these data expand the results of our study , in which we underscored, collecting administrative data from our Institute during the years 2001-2016 and studying the prevalence of diabetes in patients with pemphigus and BP, the higher frequency of DM in BP patients during the last decade. This article is protected by copyright. Read More

    Changing prevalence of diabetes mellitus in bullous pemphigoid: It is the dipeptidyl peptidase-4 inhibitors.
    J Eur Acad Dermatol Venereol 2018 Mar 23. Epub 2018 Mar 23.
    Department of Skin and Venereal Diseases, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece.
    We read with interest the letter by Fania et al on the increased prevalence of diabetes mellitus type 2 (DM2) among patients diagnosed with bullous pemphigoid (BP) after 2007. Employing a retrospective study design the authors demonstrate an abrupt raise in the prevalence of DM2 among BP patients in relation to those with pemphigus. They conclude that this trend might stem from an increase in DM2 comorbidity among BP patients. Read More

    Blistering diseases in the mature patient.
    Clin Dermatol 2018 Mar - Apr;36(2):231-238. Epub 2017 Oct 3.
    Department of Dermatovenereology, University Hospital Center Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia. Electronic address:
    Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named "pemphigoid senilis"), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita, paraneoplastic pemphigus, pemphigus vulgaris, pemphigus foliaceus, linear IgA dermatosis, and dermatitis herpetiformis. Read More

    Paraneoplastic Pemphigus Associated With Castleman Disease Detected by 18F-FDG PET/CT.
    Clin Nucl Med 2018 Mar 20. Epub 2018 Mar 20.
    A 36-year-old woman suffering from oral ulcerations, skin rash, and dyspnea was suspected of having paraneoplastic pemphigus. An F-FDG PET/CT was performed to detect the underlying neoplasms and revealed an F-FDG-avid mass in the lower right retroperitoneum. The mucocutaneous lesions and dyspnea were relieved after the tumor excision, and the pathology indicated Castleman disease. Read More

    Chronic Paronychia and Onychomadesis in Pemphigus Vegetans: An Unusual Presentation in a Rare Autoimmune Disease.
    Case Rep Med 2018 11;2018:5980937. Epub 2018 Jan 11.
    Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
    Pemphigus vegetans is a rare variant of deep acantholytic pemphigus which usually presents with vesiculobullous rash and vegetative plaques on the folds. We report a case of pemphigus vegetans patient who presented with rashes on tips of fingers and toes resembling paronychia and onychomadesis that misled the diagnosis for months. The final diagnosis of Hallopeau-type pemphigus vegetans was made based on histopathology and direct immunofluorescence studies. Read More

    [Curative effect of ozone hydrotherapy for pemphigus].
    Zhong Nan Da Xue Xue Bao Yi Xue Ban 2018 Feb;43(2):152-156
    Department of Dermatology, Second Affiliated Hospital, Kunming Medical University, Kunming 650101, China.
    Objective: To determine clinical curative effects of ozone therapy for pemphigus vulgaris.
 Methods: Ozone hydrotherapy was used as an aid treatment for 32 patients with pemphigus vulgaris. The hydropathic compression of potassium permanganate solution for 34 patients with pemphigus vulgaris served as a control. Read More

    Pemphigus Vegetans Confined to the Face and Scalp.
    Skinmed 2018 1;16(1):60-61. Epub 2018 Feb 1.
    Department of Dermatology, Habib Thameur Hospital, Tunis, Tunisia.
    A 58-year-old man presented to our department with multiple chronic vegetant plaques on the face and scalp that had been present for 4 months. The plaques were well-defined hypertrophic areas, measuring between 3 cm × 3 cm and 3 cm × 4 cm, that were located mainly on the face symmetrically and on the forehead (Figure 1). The patient did not report itching or pain. Read More

    Regulatory effects of Nr4a2 on Th2 cells from patients with pemphigus vulgaris.
    Oncotarget 2018 Feb 1;9(13):11258-11267. Epub 2018 Feb 1.
    Department of Dermatology, Hunan Key Laboratory of Medical Epigenomics, The Second Xiangya Hospital of Central South University, Changsha 410011, China.
    Pemphigus vulgaris is an autoimmune blistering disease characterized by a loss of epidermal cell-cell adhesion caused by anti-desmoglein (Dsg) autoantibodies. The pathogenesis of PV remains unclear. However, abnormal frequency and function of Th2 cells are believed to contribute to PV. Read More

    Clinical, immunological profile and follow up of patients with pemphigus: A study from India.
    Indian J Dermatol Venereol Leprol 2018 Mar 12. Epub 2018 Mar 12.
    Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
    Background: Pemphigus has a protracted course and multiple factors influence its prognosis. The objective of this study was to describe the epidemiology and clinical profile of pemphigus patients and to study its influence on treatment end points.

    Methods: This was a retrospective chart review done in an Indian tertiary care hospital from December 1991 to December 2013. Read More

    Soluble Fas Ligand Is Essential for Blister Formation in Pemphigus.
    Front Immunol 2018 26;9:370. Epub 2018 Feb 26.
    Laboratory of Cutaneous Biology, Department of Surgical, Medical, Dental and Morphological Sciences, University of Modena and Reggio Emilia, Modena, Italy.
    Pemphigus is a blistering disease characterized by pemphigus autoantibodies (PVIgG) directed mostly against desmogleins (Dsgs), resulting in the loss of keratinocyte adhesion (acantholysis). Yet, the mechanisms underlying blister formation remain to be clarified. We have shown previously that anti-Fas ligand (FasL) antibody (Ab) prevents PVIgG-induced caspase-8 activation and Dsg cleavage in human keratinocytes, and that sera from pemphigus patients contain abnormally increased levels of FasL. Read More

    Paraneoplastic Pemphigus Associated with B-cell Chronic Lymphocytic Leukemia Treated with Ibrutinib and Rituximab.
    Intern Med 2018 Mar 9. Epub 2018 Mar 9.
    Department of Hematology, National Cancer Center Hospital, Japan.
    Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease associated with an underlying malignancy, and its prognosis is poor. We herein report the first patient with B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL)-associated PNP successfully treated with the Bruton's tyrosine kinase inhibitor ibrutinib and rituximab. Although his PNP lesions did not improve with ibrutinib monotherapy, the combination of ibrutinib and rituximab was effective against B-CLL/SLL-associated PNP. Read More

    Autologous platelet-rich plasma and triamcinolone acetonide intralesional injection in the treatment of oral erosions of pemphigus vulgaris: a pilot study.
    Arch Dermatol Res 2018 Mar 10. Epub 2018 Mar 10.
    Department of Dermatology, Cairo University School of Medicine, Kasr Al Ainy hospital, Al-Manial, Cairo, PO 11956, Egypt.
    Oral erosions of pemphigus vulgaris (PV) represent a therapeutic challenge. In this work we compared intralesional injection of steroids (ILS) and autologous platelet-rich plasma (PRP) treatment of oral erosions of PV. In this split-mouth randomized double blind study, one side of the buccal mucosa of 11 PV patients was injected with 10 mg/ml triamcinolone and the opposite side was injected with 1 millilitre of autologous PRP every 14 days for 3 months. Read More

    Risk factors for lethal outcome in patients with pemphigus: a retrospective cohort study.
    Eur J Dermatol 2018 Feb;28(1):26-37
    Department of Dermatology, Rambam Health Care Campus,, Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
    Although pemphigus is associated with increased mortality, little is known regarding factors that influence prognosis. To identify prognostic factors for mortality at five- and 10-year periods after an initial diagnosis of pemphigus. A retrospective cohort study was performed. Read More

    Targeting IgE Antibodies by Immunoadsorption in Atopic Dermatitis.
    Front Immunol 2018 19;9:254. Epub 2018 Feb 19.
    Department of Dermatology, University of Lübeck, Lübeck, Germany.
    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. Read More

    Effectiveness and Safety of Rituximab in Recalcitrant Pemphigoid Diseases.
    Front Immunol 2018 19;9:248. Epub 2018 Feb 19.
    Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
    Introduction: Rituximab (RTX) is a monoclonal antibody targeting CD20, a transmembrane protein expressed on B cells, causing B cell depletion. RTX has shown great efficacy in studies of pemphigus vulgaris, but data of pemphigoid diseases are limited.

    Objective: To assess the effectiveness and safety of RTX in pemphigoid diseases. Read More

    Oral pemphigus.
    G Ital Dermatol Venereol 2018 Mar 6. Epub 2018 Mar 6.
    Dipartimento Chirurgico, Medico, Odontoiatrico e di Scienze Morfologiche con Interesse Trapiantologico, Oncologico e di Medicina Rigenerativa, Università degli Studi di Modena e Reggio Emilia, Modena, Italy.
    Involvement of the oral mucosa in patients affected by Pemphigus Vulgaris (PV), Paraneoplastic, IgA Pemphigus and in some cases of Iatrogenic Pemphigus is common, and precede skin lesions in the majority of cases. Intraepidermal bullae are caused by acantholysis, induced by IgG autoantibodies directed against the desmosomes and the domain of numerous keratinocytes self-antigens desmogleins (namely cadherins), thus supporting the autoimmune nature of the disease. Apoptosis may contribute to the acantholysis. Read More

    COPD and lung cancer in patients with pemphigus- a population based study.
    Respir Med 2018 Mar 7;136:93-97. Epub 2018 Feb 7.
    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: Recent evidence indicates that autoimmunity may contribute to the pathogenesis of chronic obstructive pulmonary disease (COPD). COPD was observed at higher frequency in patients with several autoimmune diseases. The association between pemphigus and COPD has not been evaluated in the past. Read More

    Exploring the in situ expression of vascular endothelial growth factor and endoglin in pemphigus foliaceus variants and pemphigus vulgaris.
    J Eur Acad Dermatol Venereol 2018 Feb 28. Epub 2018 Feb 28.
    Department of Dermatology, University of São Paulo Medical School, São Paulo, São Paulo, Brazil.
    Background: Erythroderma is a severe manifestation of pemphigus foliaceus (PF), a blistering disease mediated by IgG autoantibodies against desmoglein 1. Increasing evidence supports the contribution of angiogenic mediators in the pathogenesis of erythroderma.

    Objective: To evaluate the in situ expression of vascular endothelial growth factor (VEGF) and endoglin in patients with PF with erythroderma. Read More

    Seborrheic Pemphigus, Antigen Mimicry and the Subsequent-Wrong Diagnostic and Therapeutic Approach?
    Open Access Maced J Med Sci 2018 Jan 13;6(1):128-131. Epub 2018 Jan 13.
    Medical Institute of Ministry of Interior (MVR-Sofia), Department of Dermatology, Venereology and Dermatologic Surgery, Sofia, Bulgaria.
    It is well-established that drugs could be leading cause of occurrence of numerous diseases, including pemphigus, being either inducer or triggering factor of the autoimmunity. Despite medications, it should be kept in mind that chronic or acute infections are also capable of being a trigger in various types of cutaneous eruptions, including pemphigus. The rapidly obtained and uncompleted history for accompanied medication leads to general mistakes in the subsequent treatment approach, as the first step in such cases is discontinuation of the drug-inductor. Read More

    A New Classification System for IgG4 Autoantibodies.
    Front Immunol 2018 12;9:97. Epub 2018 Feb 12.
    Institute of Neurology, Medical University of Vienna, Vienna, Austria.
    IgG4 autoimmune diseases are characterized by the presence of antigen-specific autoantibodies of the IgG4 subclass and contain well-characterized diseases such as muscle-specific kinase myasthenia gravis, pemphigus, and thrombotic thrombocytopenic purpura. In recent years, several new diseases were identified, and by now 14 antigens targeted by IgG4 autoantibodies have been described. The IgG4 subclass is considered immunologically inert and functionally monovalent due to structural differences compared to other IgG subclasses. Read More

    Pemphigus group: overview, epidemiology, mortality, and comorbidities.
    Immunol Res 2018 Apr;66(2):255-270
    Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel.
    Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. It is characterized by the production of pathogenic autoantibodies directed against different proteins of the desmosome, leading histologically to intraepidermal cleavage, and clinically to vesicles and erosions on the epithelium of the mucous membranes and/or the skin. Read More

    Influence of cigarette smoking on pemphigus - a systematic review and pooled analysis of the literature.
    J Eur Acad Dermatol Venereol 2018 Feb 25. Epub 2018 Feb 25.
    Department of Dermatology, University of Lübeck, Lübeck, Germany.
    Epidemiological evidence suggests that smoking cigarettes may be beneficial in pemphigus, but no systematic evaluation exists to corroborate this assumption. Therefore, a systematic literature review with pooled data analysis of the smoking status in patients with pemphigus was conducted. Electronic searches using PubMed from inception to November 2017 identified 13 reports meeting predetermined inclusion and exclusion criteria. Read More

    Pénfigo foliáceo agravado por fármacos en un paciente con psoriasis, con buena respuesta a adalimumab.
    Dermatol Online J 2017 Oct 15;23(10). Epub 2017 Oct 15.
    Servicio de Dermatología. Hospital Universitario Rey Juan Carlos. Móstoles. Madrid. España.
    El pénfigo foliáceo es una enfermedad autoinmune ampollosa debida a la producción de autoanticuerpos frente a la desmogleína 1. Presentamos el caso de un paciente con psoriasis y pénfigo foliáceo agravado por enalapril y amlodipino, con buena respuesta de ambas patologías a la terapia con adalimumab.Pemphigus foliaceus is a blistering autoimmune disease related to the production of autoantibodies against desmoglein 1. Read More

    Unusually extensive scalp ulcerations manifested in pemphigus erythematosus.
    Dermatol Online J 2018 Jan 15;24(1). Epub 2018 Jan 15.
    University of Illinois College of Medicine, Chicago, Illinois.
    Senear-Usher Syndrome, or pemphigus erythematosus, is an autoimmune skin blistering disorder with an overlapping clinical presentation of pemphigus foliaceus and lupus erythematosus. Lesions typically involve the scalp, face, and upper chest or back. This case study focuses on a patient who presentedwith progressive scalp ulcers, hyperpigmentation, and eroded plaques with overlying hemorrhagic crust. Read More

    Clinical resolution of pemphigus vulgaris on rituximab.
    Dermatol Online J 2017 Sep 15;23(9). Epub 2017 Sep 15.
    Department of Dermatology, Stony Brook University School of Medicine, New York, New York.
    Although significant progress has been made for the treatment of pemphigus vulgaris (PV) with rituximab (RTX), a consensus remains to be determined for standard treatment protocol, regarding optimal dosing, infusion regimen, and use of concomitant immunotherapy to achieve safe, effective, and rapid clinical response. We describe a patient with pemphigus vulgaris treated with high dose rituximab with the rheumatoid arthritis protocol along with intravenous immunoglobulin therapy. This case provides evidence towards the growing body of research needed to modify and improve treatment for pemphigus using rituximab. Read More

    Acquired bullous acrodermatitis enteropathica as a histologic mimic of pemphigus foliaceus in a patient on parenteral nutrition.
    Dermatol Online J 2017 Jul 15;23(7). Epub 2017 Jul 15.
    Department of Dermatology, Hofstra Northwell School of Medicine, New Hyde Park, New York. vsharon@
    Acquired zinc deficiency can develop as a consequence of poor nutritional intake or from dependence on total parenteral nutrition. Acquired zinc deficiency dermatitis classically manifests with erosions and scaly plaques in a periorificial and acral distribution. We present a case of a woman on parenteral nutrition who presented with bullous acrodermatitis mimicking pemphigus foliaceus histopathologically. Read More

    Association Between Pemphigus and Neurologic Diseases.
    JAMA Dermatol 2018 Mar;154(3):281-285
    Department of Quality Measurements and Research, Chief Physician's Office, Clalit Health Services, Tel Aviv, Israel.
    Importance: The association between pemphigus and neurologic diseases was not evaluated systematically in the past. In a recent uncontrolled cross-sectional study, Parkinson disease was found to be significantly associated with pemphigus; in the same study, epilepsy had a nonsignificant association with pemphigus. Several case reports have suggested that pemphigus coexists with multiple sclerosis and dementia. Read More

    Coexistent Solid Malignancies in Pemphigus: A Population-Based Study.
    JAMA Dermatol 2018 Apr;154(4):435-440
    Department of Quality Measurements and Research, Chief Physician's Office, Clalit Health Services, Tel Aviv, Israel.
    Importance: The association of pemphigus vulgaris and pemphigus foliaceus with comorbid malignancies is yet to be firmly established.

    Objective: To estimate the association between pemphigus and a wide range of nonhematologic malignancies using one of the largest cohorts of patients with pemphigus to date.

    Design, Setting, And Participants: For this cross-sectional study, we used the computerized database of Clalit Health Services, the largest public health care provider organization in Israel insuring 4. Read More

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