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    Successful Treatment of a Bullous Pemphigoid Patient with Rituximab Who Was Refractory to Corticosteroid and Omalizumab Treatments.
    Case Rep Dermatol 2017 Jan-Apr;9(1):38-44. Epub 2017 Feb 10.
    Akdeniz University Faculty of Medicine, Dermatology and Venereology Department, Antalya, Turkey.
    Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP). Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. Read More

    Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease?
    Hematol Oncol 2017 Apr 11. Epub 2017 Apr 11.
    Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
    Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. Read More

    Comparison of Immunofluorescence and Desmoglein Enzyme-linked Immunosorbent Assay in the Diagnosis of Pemphigus: A Prospective, Cross-sectional Study in a Tertiary Care Hospital.
    Indian J Dermatol 2017 Mar-Apr;62(2):171-177
    Department of Dermatology, Kasturba Medical College, Manipal University, Manipal, Karnataka, India.
    Background: Pemphigus is an acquired immunobullous disorder in which antibodies are directed against epidermal cadherins. Despite the commercial availability and less cost of enzyme-linked immunosorbent assays (ELISAs) to detect antidesmoglein 1 (Dsg1) and anti-Dsg3, immunofluorescence is still widely used for confirmation of diagnosis.

    Aims: (1) To compare the usefulness of indirect immunofluorescence (IIF) and ELISA tests in the diagnosis of pemphigus. Read More

    Effect of intravenous pulse dexamethasone versus daily oral prednisolone on bone mineral density in dermatology patients: Is it a site-specific response?
    Indian J Dermatol Venereol Leprol 2017 Apr 10. Epub 2017 Apr 10.
    Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Background: The use of glucocorticoids in various forms of administration is complicated by their systemic side effects. Although intravenous pulse therapy is considered to have lesser systemic side effects, there are few studies in literature comparing the effects of intravenous pulse glucocorticoids versus oral daily glucocorticoids on bone mineral density.

    Aim: To compare the effects of intravenous pulse glucocorticoids and oral daily glucocorticoids on bone mineral density with the aim of finding any site-specific osteopenic side effect. Read More

    [Clinicopathological features of acantholytic mammary Paget's disease: a report of 28 cases].
    Zhonghua Yi Xue Za Zhi 2017 Apr;97(14):1076-1078
    Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
    Objective: To investigate the clinicopathological features of acantholytic mammary Paget's disease (AMPD). Methods: From January, 2010 to October, 2016, a total of 28 patients were diagnosed as AMPD in the Department of Dermatology, Peking Union Medical College Hospital. The clinical and histopathological data of these patients were analyzed retrospectively. Read More

    DRUG-INDUCED ORAL ULCERATIONS: CASE REPORT.
    Acta Clin Croat 2016 06;55(2):334-7
    A 70-year-old patient was admitted to the Department of Oral Medicine for multiple oral ulcerations on the left buccal mucosa, around 0.5 cm in diameter, as well as on the gingiva. Otherwise, the patient suffered from chronic lymphocytic leukemia, hypogammaglobulinemia, chronic renal insufficiency, with complete afunction of the right kidney, asthma, hypertension, gastritis and prostate hyperplasia. Read More

    Increased miR-424-5p expression in peripheral blood mononuclear cells from patients with pemphigus.
    Mol Med Rep 2017 Apr 3. Epub 2017 Apr 3.
    Department of Dermatology, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong 510515, P.R. China.
    Pemphigus is an autoimmune disease that causes blisters and erosions in the skin and mucous membranes. The development of pemphigus is associated with the imbalance of T‑cell and humoral responses. MicroRNAs (miRNAs) can regulate many cell functions. Read More

    Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy.
    Ann Dermatol 2017 Apr 24;29(2):219-222. Epub 2017 Mar 24.
    Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
    Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Read More

    Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016.
    J Invest Dermatol 2017 Apr 5. Epub 2017 Apr 5.
    Institute of Anatomy and Cell Biology, Ludwig-Maximilians-Universität, Munich, Germany.
    Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases. Read More

    Augenbeteiligung beim Pemphigus vulgaris - retrospektive Studie an einer großen spanischen Kohorte.
    J Dtsch Dermatol Ges 2017 Apr;15(4):396-404
    Hospital Gregorio Marañon of Madrid, Spanien.
    Hintergrund Und Ziele: Es gibt nur wenige Berichte zur Beteiligung der okulären/periokulären Region beim Pemphigus vulgaris (PV). Ziel der vorliegenden Studie war es, das Krankheitsbild des okulären PV (OPV) zu untersuchen und seinen prognostischen Wert zu definieren.

    Patienten Und Methodik: Zwischen 1985 und 2014 wurden insgesamt 167 Patienten mit Pemphigus vulgaris an vier tertiären spanischen Krankenhäusern behandelt. Read More

    Successful treatment of pemphigus foliaceus in a Berrichon du Cher ram with methylprednisolone acetate.
    Vet Dermatol 2017 Apr 4. Epub 2017 Apr 4.
    University Clinic for Ruminants of the University of Veterinary Medicine Vienna, Veterinärplatz 1, 1210, Vienna, Austria.
    Background: Pemphigus foliaceus is a severe, autoimmune blistering skin disease, which is described in humans and some animal species. In small ruminants pemphigus foliaceus has rarely been described and, to the best of the authors' knowledge, little information is available about successful treatment in sheep.

    Aim: This case report describes a Berrichon du Cher ram with the presumed diagnosis of pemphigus foliaceus. Read More

    Efficacy of Triaging Direct Immunofluorescence in Intraepidermal Bullous Dermatoses.
    Am J Dermatopathol 2017 Mar 24. Epub 2017 Mar 24.
    *Department of Pathology, University of South Dakota-Sanford School of Medicine, Sioux Falls, SD; and †Departments of Pathology and Dermatology, Cleveland Clinic Foundation, Cleveland, OH.
    Background: Direct immunofluorescence (DIF) is considered pivotal in diagnosing autoimmune blistering diseases. Our goal was to examine the necessity of DIF in intraepidermal bullous cases, of which pemphigus vulgaris (PV) is the prototype.

    Methods: Sixty-six cases from 2010 to 2014 submitted for DIF with an intraepidermal blistering disease listed in the differential diagnosis were reviewed by 2 board-certified dermatopathologists to see if they would order DIF based on routine histologic findings. Read More

    Low-dose rituximab as an adjuvant therapy in pemphigus.
    Indian J Dermatol Venereol Leprol 2017 May-Jun;83(3):317-325
    Department of Dermatology, NHL Medical College, VS Hospital, Ahmedabad, Gujarat, India.
    Background: Pemphigus is a chronic autoimmune blistering disease where systemic steroids and immunosuppressants are the mainstay of therapy, but long-term treatment with these agents is associated with many side effects. Rituximab, a chimeric monoclonal anti-CD20 antibody, in low doses has shown efficacy as an adjuvant to reduce the dose of steroids.

    Aim: To study the clinical efficacy and safety of low-dose rituximab as an adjuvant therapy in pemphigus. Read More

    [Thymoma and autoimmune diseases].
    Rev Med Interne 2017 Mar 29. Epub 2017 Mar 29.
    Service de médecine interne, hospices civils de Lyon, hôpital de la Croix-Rousse, université Claude-Bernard - Lyon 1, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.
    The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Read More

    Trichilemmal cystis in metastatic melanoma: a case report.
    Exp Oncol 2017 03;39(1):86-87
    Department of Dermatology, Department of Critical Care Medicine and Surgery, University of Florence, Firenze 50121, 50132, Italy.
    The malignant melanoma is a neoplasm associated with a wide variety of cutaneous paraneoplastic syndromes, as dermatomyositis, systemic sclerosis, paraneoplastic pemphigus. We describe a case of four multiple trichilemmal cystis arising on frontal region in the same patient with brain metastasis and unknown primary melanoma and discuss their relationship. Read More

    Cassia fistula: A remedy from Traditional Persian Medicine for treatment of cutaneous lesions of pemphigus vulgaris.
    Avicenna J Phytomed 2017 Mar-Apr;7(2):107-115
    Department of Traditional Persian Medicine, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
    Objective: Pemphigus is a rare autoimmune disease that may be fatal without proper medical intervention. It is a blistering disease that involves both the skin and mucus membranes, in which the most important causes of death comprise superimposed opportunistic infections and complications of long-term high-dose corticosteroid therapy or prolonged consumption of immune suppressant drugs. Skin lesions are the most important sources of infection, and any local treatment decreasing the healing time of lesions and reducing the total dosage of drugs is favorable. Read More

    Nasal, oral, and pharyngolaryngeal manifestations of pemphigus vulgaris: Endoscopic ororhinolaryngologic examination.
    Ear Nose Throat J 2017 Mar;96(3):120-127
    Department of Otolaryngology, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe-shi, Saitama 350-8550, Japan.
    Pemphigus vulgaris is an autoimmune blistering disorder that involves the skin and mucous membranes. Few reports have described nasal and oropharyngolaryngeal lesions in pemphigus vulgaris using an endoscopic ororhinolaryngologic examination. We retrospectively reviewed the clinical records of 11 patients with pemphigus vulgaris between 2001 and 2013 with respect to their symptoms, lesion sites, lesion features, and treatments received. Read More

    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.
    Lancet 2017 Mar 22. Epub 2017 Mar 22.
    Department of Dermatology, Rouen University Hospital and INSERM U1234, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France.
    Background: High doses of corticosteroids are considered the standard treatment for pemphigus. Because long-term corticosteroid treatment can cause severe and even life-threatening side-effects in patients with this disease, we assessed whether first-line use of rituximab as adjuvant therapy could improve the proportion of patients achieving complete remission off-therapy, compared with corticosteroid treatment alone, while decreasing treatment side-effects of corticosteroids.

    Methods: We did a prospective, multicentre, parallel-group, open-label, randomised trial in 25 dermatology hospital departments in France (Ritux 3). Read More

    Case of bullous pemphigoid coexisting with anti-desmoglein autoantibodies.
    J Dermatol 2017 Mar 24. Epub 2017 Mar 24.
    Department of Dermatology, Shimane University Faculty of Medicine, Izumo, Japan.
    A 79-year-old Japanese woman had clinical and histopathological features of bullous pemphigoid, while direct immunofluorescence test revealed C3 and immunoglobulin G depositions in the lower cell surfaces of the epidermis in addition to those in the dermoepidermal junction. Chemiluminescent enzyme immunoassays were positive for desmoglein-1 and -3 antibodies in addition to anti-BP180 antibodies. In an immunoblotting study, antibodies against both 180-kDa bullous pemphigoid antigen and 130-kDa pemphigus vulgaris antigen were detected. Read More

    Ocular involvement in pemphigus vulgaris - a retrospective study of a large Spanish cohort.
    J Dtsch Dermatol Ges 2017 Apr 23;15(4):396-403. Epub 2017 Mar 23.
    Hospital Gregorio Marañon of Madrid, Spain.
    Background And Objectives: Ocular/periocular involvement in pemphigus vulgaris (OPV) has rarely been reported. The objective of the present study was to investigate the pattern of OPV and define the prognostic value of its manifestation.

    Patients And Methods: From 1985 to 2014, a total of 167 patients with pemphigus vulgaris (PV) were treated at four tertiary Spanish hospitals. Read More

    Immunopathogenic Oral Diseases: An Overview 
Focusing on Pemphigus Vulgaris and Mucous Membrane Pemphigoid.
    Oral Health Prev Dent 2017 ;15(2):177-182
    Pemphigus vulgaris, mucosal pemphigoid (mucous membrane pemphigoid), lichen planus, discoid lupus erythematosus and erythema multiforme are a group of immune-mediated mucocutaneous disorders characterised clinically by the formation of blisters, erosions or ulcers. The oral mucosa is often affected, and sometimes the disease is limited to the mouth. The target antigens, autoreactive immune responses, microscopic features, treatment and prognosis vary from one disease to the other. Read More

    Th1/Th17-Related Cytokines and Chemokines and Their Implications in the Pathogenesis of Pemphigus Vulgaris.
    Mediators Inflamm 2017 22;2017:7151285. Epub 2017 Feb 22.
    Laboratory of Immunology, Federal University of Triângulo Mineiro, Uberaba, MG, Brazil.
    Pemphigus vulgaris (PV) is an autoimmune disease characterized by the presence of IgG autoantibodies against desmoglein-3. Despite the variety of findings, the chemokine and cytokine profiles that characterize the immune response in the disease are still poorly explored. Thus, 20 PV patients and 20 controls were grouped according to gender, ethnicity, place of residence, and clinical parameters of the disease. Read More

    Is photodynamic therapy a relevant therapeutic option in refractory benign familial pemphigus (Hailey-Hailey disease)? A series of eight patients.
    J Dermatolog Treat 2017 Apr 2:1-5. Epub 2017 Apr 2.
    a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
    Introduction/background: Treatment of benign familial pemphigus or Hailey-Hailey disease (HHD), a rare inherited condition associated with a significant impairment of quality of life, is often challenging and disappointing with frequent relapses and infectious complications. Topical photodynamic therapy (PDT) may offer new perspectives in this difficult setting.

    Material And Methods: Eight patients with long-lasting HHD lesions refractory to multiple treatments were treated on at least one involved site with PDT using methyl-amino levulinate with a standardized protocol of three sessions of irradiation separated by 3-week intervals. Read More

    Longitudinal Tracking of Autoantibody Levels in a Pemphigus Vulgaris Patient: Support for a Role of Anti-Desmoglein 1 Autoantibodies as Predictors of Disease Progression.
    J Drugs Dermatol 2017 Feb;16(2):135-139
    Anti-desmoglein (Dsg) 1 and -Dsg3 antibody titers have an established role in the diagnosis of the autoimmune blistering skin disease pemphigus vulgaris (PV). However, their usefulness for disease monitoring has been controversial. A recent large-scale immunoprofiling study by our group indicated that anti-Dsg1 levels may be a better predictor of disease activity than anti-Dsg3 levels, with declining levels predicting progression from active phase of disease to early remission, irrespective of lesional subtypes. Read More

    Pemphigus Vulgaris and Eosinophilic Esophagitis in a 13-Year-Old Boy: Case Report and Review of the Literature.
    Pediatr Dermatol 2017 Mar;34(2):e80-e84
    Discipline of Paediatrics, University of Adelaide, Adelaide, South Australia, Australia.
    This case report presents a 13-year-old boy referred to the Department of Paediatric Dentistry, Women's and Children's Hospital, Adelaide, South Australia, Australia, with a 5-week history of severe oral ulcerations and significant weight loss of unknown origin. The diagnosis of pemphigus vulgaris was made after histologic and immunofluorescent examination of an intraoral deep incisional biopsy, with eosinophilic esophagitis also diagnosed during the initial upper gastrointestinal endoscopy. The association between pemphigus vulgaris and eosinophilic esophagitis in this case, although previously unreported, is explicable on the basis of dysregulation of desmoglein 1 (DSG1). Read More

    Successful use of Bruton's kinase inhibitor, ibrutinib, to control paraneoplastic pemphigus in a patient with paraneoplastic autoimmune multiorgan syndrome and chronic lymphocytic leukaemia.
    Australas J Dermatol 2017 Mar 13. Epub 2017 Mar 13.
    Department of Dermatology, Royal North Shore Hospital, Sydney, New South Wales, Australia.
    We present the case of a 51-year-old man who developed paraneoplastic pemphigus (PNP) in the context of chronic lymphocytic leukemia (CLL). His CLL was successfully controlled with ibrutinib. Concurrently, there was significant improvement of his PNP, suggesting that ibrutinib may be a very useful addition to the treatment options in this potentially life-threatening autoimmune disorder. Read More

    Setting the target for pemphigus vulgaris therapy.
    JCI Insight 2017 Mar 9;2(5):e92021. Epub 2017 Mar 9.
    Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
    Despite the rising incidence of autoimmunity, therapeutic options for patients with autoimmune disease still rely on decades-old immunosuppressive strategies that risk severe and potentially fatal complications. Thus, novel therapeutic approaches for autoimmune diseases are greatly needed in order to minimize treatment-related toxicity. Such strategies would ideally target only the autoreactive immune components to preserve beneficial immunity. Read More

    Pemphigus vulgaris and pemphigus foliaceus determined by CD86 and CTLA4 polymorphisms.
    Clin Dermatol 2017 Mar - Apr;35(2):236-241. Epub 2016 May 30.
    Institute of Microbiology and Immunology, School of Medicine, University of Belgrade, Belgrade, Serbia. Electronic address:
    Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are rare autoimmune blistering diseases with presumed T-cell-dependent pathology. Activation of naïve T cells is dependent on antigen recognition, subsequent signaling through the T-cell receptor complex (signal 1), and various other interactions of T cells with antigen presenting cells that may be collectively designated as signal 2, which is unconditionally required for T-cell activation both in response to infection and to autoantigens. Among the best described interactions contributing to signal 2 are those mediated by B7 family molecules, such as CD80 and CD86 with their ligands; CD28, providing activation signals; and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4), conferring inhibition. Read More

    Accuracy of molecular diagnostics in pemphigus and bullous pemphigoid: comparison of commercial and modified mosaic indirect immunofluorescence tests as well as enzyme-linked immunosorbent assays.
    Postepy Dermatol Alergol 2017 Feb 7;34(1):21-27. Epub 2017 Feb 7.
    Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.
    Introduction: Pemphigus and bullous pemphigoid (BP) are identified by autoantibodies (abs) against desmoglein 1, 3 (DSG1/3) and BP180/BP230, respectively. A novel mosaic to indirect immunofluorescence (IIF) using purified BP180 recombinant proteins spotted on slide and transfected cells expressing BP230, DSG1, DSG3 is available. The commercial (IgG detection) and modified (IgG4 detection) mosaic for indirect immunofluorescence (IIFc - IIF commercial, IIFm - IIF modified) and IgG ELISAs were evaluated in pemphigus and bullous pemphigoid (BP) molecular diagnostics. Read More

    Mimickers of classic acantholytic diseases.
    J Dermatol 2017 Mar;44(3):232-242
    Department of Dermatology and Section of Dermatopathology, Boston University School of Medicine, Boston, Massachusetts, USA.
    Acantholysis is a commonly encountered histological pattern which typically generates a differential of the pemphigus variants, Hailey-Hailey, Darier's and Grover's diseases. In addition to these diseases, the dermatologist and dermatopathologist must be aware of entities that mimic classic acantholytic dermatoses and of rare disease variants, which are characterized by acantholysis. Read More

    A resistant case of pemphigus gestationis successfully treated with cyclosporine.
    Interv Med Appl Sci 2016 Mar;8(1):20-22
    Department of Obstetrics and Gynecology, Ankara Numune Education and Research Hospital , Ankara , Turkey.
    Pemphigoid gestationis (PG) is a rare autoimmune blistering disease of pregnancy caused by antibasement membrane zone auto-antibodies. The usual clinical findings are multiple pruritic urticarial papules and plaques, target lesions, vesicles, and blisters that occur during the second and third trimesters of pregnancy or in the immediate postpartum period. The disease is often treated with topical corticosteroids and oral antihistaminics. Read More

    [Dermatoses and herpes superinfection: A retrospective study of 34 cases].
    Ann Dermatol Venereol 2017 Mar 24;144(3):176-181. Epub 2017 Feb 24.
    Service de dermatologie, hôpital Cochin, université Paris Descartes, Assistance publique-Hôpitaux de Paris (AP-HP), pavillon Tarnier, 89, rue d'Assas, 75006 Paris, France. Electronic address:
    Background: Although varicelliform Kaposi eruption is a well-known complication of dermatoses, it has not been widely investigated.

    Aim: To investigate features of dermatoses and herpes superinfection in patients hospitalized in a dermatology department.

    Patients And Methods: We performed a single-centre, retrospective study between 2008 and 2014 that included cases of Kaposi varicelliform eruptions defined by positive PCR of an unconventional site of herpetic recurrence in a setting of active dermatitis. Read More

    Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: A case report.
    Medicine (Baltimore) 2017 Feb;96(8):e6184
    Department of Hematology, The First Affiliated Hospital of Chongqing University of Medical Sciences, Chongqing, China.
    Rationale: Paraneoplastic pemphigus (PNP) is an autoimmune syndrome associated with neoplasms. The treatment approach principally includes suppressing the immunity, but its therapeutic effect is not satisfying.

    Patient Concerns: We report a case of paraneoplastic pemphigus linked to chronic lymphocytic leukemia in a 63-year-old man. Read More

    Do serum prolactin levels correlate with antibodies against desmoglein in pemphigus vulgaris?
    Adv Biomed Res 2016 27;5:206. Epub 2016 Dec 27.
    Skin Diseases and Leishmaniasis Research Center, Department of Dermatology, Isfahan University of Medical Sciences, Isfahan, Iran.
    Background: Pemphigus vulgaris is a chronic inflammatory disease of skin, the etiology of which is not completely known. Despite the latter, anti-desmoglein antibodies play a proven role in the pathogenesis. Recent studies showed an etiologic effect for prolactin in the pemphigus vulgaris. Read More

    Utility of immunofluorescence in dermatology.
    Indian Dermatol Online J 2017 Jan-Feb;8(1):1-8
    Department of Dermatology, Kasturba Medical College, Manipal University, Manipal, Karnataka, India.
    Immunofluorescence (IF) tests have redefined our understanding of many immune-mediated skin diseases, especially autoimmune blistering diseases (AIBDs). Nomenclature of certain AIBDs (for example, linear IgA diseases and IgA pemphigus) has been done based solely on the finding of tissue-bound immunoreactants as detected by IF tests. Direct and indirect are the two major types of IF tests; they are not only useful in the diagnosis but also guide the clinician in the treatment at least in certain AIBDs, as the titer of circulating antibodies as detected by IF reflects the disease activity. Read More

    Autoantibodies other than Anti-desmogleins in Pemphigus Vulgaris Patients.
    Indian J Dermatol 2017 Jan-Feb;62(1):47-51
    Department of Medical Biochemistry, Cairo University, Cairo, Egypt.
    Background: Pemphigus vulgaris (PV) is an immunoglobulin G-mediated autoimmune bullous skin disease. Nonorgan-specific antibodies were detected in Tunisian and Brazilian pemphigus patients with different prevalence.

    Materials And Methods: Fifty PV patients and fifty controls were screened for antinuclear antibodies (ANAs), anti-smooth muscle antibodies (ASMAs), anti-parietal antibodies (APAs), anti-mitochondrial antibodies, and Anti-nuclear cytoplasmic antibodies (ANCA) by indirect immunofluorescence. Read More

    Role of FOXP3 gene polymorphism in the susceptibility to Tunisian endemic Pemphigus Foliaceus.
    Immunol Lett 2017 Apr 16;184:105-111. Epub 2017 Feb 16.
    Immunology Department, Habib Bourguiba Hospital, University of Sfax, Sfax, Tunisia. Electronic address:
    Objective: Forkhead box P3 (FOXP3) is an essential and crucial transcription factor of regulatory T-cells. Genetic polymorphisms in the promoter region of FOXP3 gene may alter the gene expression level and, therefore, contribute to several autoimmune diseases susceptibility. We aimed to investigate the possible role of genetic variants of four SNPs (rs3761547, rs3761548, rs3761549 and rs2294021) and a (GT)n microsatellite located in FOXP3 gene in the susceptibility to Tunisian Pemphigus Foliaceus (PF). Read More

    Pemphigus, a pathomechanism of acantholysis.
    Australas J Dermatol 2017 Feb 17. Epub 2017 Feb 17.
    Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan.
    Autoantibodies to the desmosomal proteins desmoglein 1 and 3 cause pemphigus foliaceus and pemphigus vulgaris, which are characterised by keratinocyte dissociation (acantholysis) and intraepidermal blister formation. The passive transfer of pathogenic anti-desmoglein antibodies induces blisters in mice in vivo and the loss of keratinocyte adhesion in vitro. The pathogenetic mechanisms of acantholysis due to anti-desmoglein autoantibodies are not fully understood. Read More

    Low-dose rituximab and concurrent adjuvant therapy for pemphigus: Protocol and single-centre long-term review of nine patients.
    Australas J Dermatol 2017 Feb 17. Epub 2017 Feb 17.
    Department of Dermatology, Royal Melbourne Hospital, Melbourne, Victoria, Australia.
    Pemphigus is an autoimmune B-cell mediated blistering disease associated with significant morbidity and mortality. Rituximab has proven effective for the treatment of steroid-refractory pemphigus, although there is controversy over the optimum dosing protocol. Additionally, effective disease control often requires long-term immunosuppression, even in disease-free periods. Read More

    The Inpatient Burden of Autoimmune Blistering Disease in US Children: Analysis of Nationwide Inpatient Sample Data.
    Am J Clin Dermatol 2017 Apr;18(2):287-297
    Departments of Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Suite 1600, 676 N. St. Clair St., Chicago, IL, 60611, USA.
    Background: Little is known about the epidemiology of pediatric autoimmune blistering disorders (PAIBD).

    Objective: We sought to determine the inpatient burden and comorbidities of PAIBD.

    Methods: We analyzed data from the Nationwide Inpatient Sample from 2002 to 2012, which contained a representative 20% sample of all US hospitalizations. Read More

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