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    The Inpatient Burden of Autoimmune Blistering Disease in US Children: Analysis of Nationwide Inpatient Sample Data.
    Am J Clin Dermatol 2017 Feb 16. Epub 2017 Feb 16.
    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

    Mortality and differential diagnoses of villous atrophy without coeliac antibodies.
    Eur J Gastroenterol Hepatol 2017 Jan 11. Epub 2017 Jan 11.
    aFirst Department of Internal Medicine, Coeliac CentreDepartments of bClinical BiochemistrycBiometry and Clinical Epidemiology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
    Objective: Villous atrophy (VA) of the small bowel is mainly related to coeliac disease (CD), whose diagnosis is made on the basis of positive endomysial/tissue transglutaminase antibodies while on a gluten-containing diet in the vast majority of patients. However, VA can also occur in other conditions whose epidemiology is little known. Our aim was to study the epidemiology and clinical features of these rare enteropathies. Read More

    Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis.
    JAMA Dermatol 2016 Dec 28. Epub 2016 Dec 28.
    Department of Dermatology, Emory University School of Medicine, Atlanta, Georgia.
    Importance: Dermatitis herpetiformis (DH) is an autoimmune blistering condition seen in the context of celiac disease. While typically managed by gluten-free diet and dapsone, treatment of DH refractory to standard treatments is not well defined.

    Observations: A man in his 80s with DH not controlled by gluten-free diet (with poor adherence), dapsone, and conventional immune-suppressing agents responded to treatment with rituximab according to the lymphoma protocol (4 weekly infusions of 375 mg/m2). Read More

    Research Techniques Made Simple: Mouse Models of Autoimmune Blistering Diseases.
    J Invest Dermatol 2017 Jan;137(1):e1-e6
    Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany. Electronic address:
    Autoimmune blistering diseases are examples of autoantibody-mediated, organ-specific autoimmune disorders. Based on a genetic susceptibility, such as a strong HLA-class II association, as yet unknown triggering factors induce the formation of circulating and tissue-bound autoantibodies that are mainly directed against adhesion structures of the skin and mucous membranes. Compared with other autoimmune diseases, especially systemic disorders, the pathogenicity of autoimmune blistering diseases is relatively well described. Read More

    Bullous Dermatosis in an End-Stage Renal Disease Patient: A Case Report and Literature Review.
    Case Rep Nephrol 2016 24;2016:6713807. Epub 2016 Nov 24.
    Division of Nephrology and Hypertension, Department of Internal Medicine, Wayne State University Detroit Medical Center, Detroit, MI, USA.
    Patients with advanced chronic kidney disease including ESRD patients may present with a wide spectrum of cutaneous abnormalities, ranging from xerosis to hyperpigmentation to severe deforming necrotizing lesions. Skin problems are not uncommon in this population of patients, with a clinical presentation that can be quite bizarre, mandating a long list of differential diagnostic possibilities, and subsequent rise of a puzzling diagnostic challenge. We describe an ESRD patient who presented with blistering, nonhealing ulcerative lesions with a diagnostic skin biopsy revealing a mixed pattern of linear IgA bullous dermatosis and dermatitis herpetiformis. Read More

    Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis.
    Acta Derm Venereol 2016 Dec 16. Epub 2016 Dec 16.
    Department of Dermatology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, 2630 Sugitani, Toyama-shi, Toyama, Japan.
    is missing (Short communication). Read More

    Cutaneous Manifestation of Food Allergy.
    Immunol Allergy Clin North Am 2017 Feb 28;37(1):217-231. Epub 2016 Oct 28.
    Division of Clinical Immunology and Allergy, Department of Pediatrics, Children's Hospital Los Angeles, 4650 Sunset Boulevard MS#75, Los Angeles, CA 90027, USA. Electronic address:
    Hypersensitivity reactions to foods can have diverse and highly variable manifestations. Cutaneous reactions, such as acute urticaria and angioedema, are among the most common manifestations of food allergy. However, cutaneous manifestations of food allergy encompass more than just IgE-mediated processes and include atopic dermatitis, contact dermatitis, and even dermatitis herpetiformis. Read More

    Are anti-epidermal transglutaminase (eTG) antibodies titre correlated with dermatitis herpetiformis lesions during the disease follow-up?
    G Ital Dermatol Venereol 2016 Nov 15. Epub 2016 Nov 15.
    Section of Dermatology, Di.S.Sal, AOU San Martino-IRCCS-IST, Genoa, Italy -
    Background: Dermatitis herpetiformis (DH) is characterized by the presence of anti-tissue transglutaminase (tTG) IgA antibodies in patient sera. In 2002, anti-epidermal transglutaminase (eTG) antibodies have been identified in DH patients. Nowadays, their role is still controversial. Read More

    Autoimmunity to heat shock proteins and vitamin D status in patients with celiac disease without associated dermatitis herpetiformis.
    J Steroid Biochem Mol Biol 2016 Oct 17. Epub 2016 Oct 17.
    Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.
    Inflammation-induced heat shock proteins (HSPs) and hypovitaminosis D have been reported to impact immune responses and to be associated with autoimmune diseases including celiac disease (CD), a gluten-sensitive enteropathy mediated by autoantibodies against tissue transglutaminase (TG2). Recently, we provided evidence for a role of autoantibodies to HSPs in patients with dermatitis herpetiformis (DH), an inflammatory skin disease induced by underlying latent CD. In this study, we aimed at investigating the humoral autoimmune response to HSPs and vitamin D status in CD patients (n=15) presenting without the cutaneous disease manifestation. Read More

    Incidence of autoimmune bullous diseases in Serbia: a 20-year retrospective study.
    J Dtsch Dermatol Ges 2016 Oct;14(10):995-1005
    Institute of Social Medicine, Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
    Background And Objectives: While most previous surveys on the clinico-epidemiological features of autoimmune bullous diseases (AIBDs) have predominantly focused on a single disease entity or just one disease group, there have been only few studies examining the incidence of various AIBDs. In the present study, we set out to determine the spectrum of AIBDs, to estimate the incidence of the most common AIBDs, and to examine their temporal trends in Central Serbia over a period of 20 years.

    Methods: We retrospectively recruited 1,161 new AIBD cases diagnosed in Central Serbia during the period from January 1991 to December 2010. Read More

    Inzidenz von bullösen Autoimmunerkrankungen in Serbien: eine retrospektive Studie über 20 Jahre.
    J Dtsch Dermatol Ges 2016 Oct;14(10):995-1006
    Institut für Sozialmedizin, Medizinische Fakultät, Universität Belgrad, Belgrad, Serbien.
    Hintergrund Und Ziele: Die meisten früheren Arbeiten zu den klinisch-epidemiologischen Merkmalen von bullösen Autoimmunerkrankungen (AIBD) konzentrierten sich vor allem auf eine einzige Krankheitsentität oder nur eine Krankheitsgruppe; nur in wenigen Studien wurde die Inzidenz verschiedener AIBD untersucht. Bei der vorliegenden Studie war es unser Ziel, das gesamte Spektrum der AIBD zu betrachten, die Inzidenz der häufigsten AIBD zu ermitteln und die zeitlichen Trends ihres Auftretens in Zentralserbien über einen Zeitraum von 20 Jahren zu untersuchen.

    Methoden: Wir rekrutierten retrospektiv 1161 AIBD-Fälle, die in Zentralserbien von Januar 1991 bis Dezember 2010 neu diagnostiziert wurden. Read More

    Extra-intestinal Manifestations of Celiac Disease: Effectiveness of the Gluten Free Diet.
    J Pediatr Gastroenterol Nutr 2016 Oct 13. Epub 2016 Oct 13.
    *Department of Pediatrics, University of Chicago Medicine Comer Children's Hospital, Chicago, IL †Department of Pediatrics, Università degli Studi di Verona, Verona, Italy.
    Objective: To evaluate the effectiveness of the GFD on extra-intestinal symptoms in pediatric and adult celiac populations at the University of Chicago (UofC).

    Methods: We conducted a retrospective chart review of the UofC Celiac Center clinic charts from January 2002 to October 2014. Demographics, serologic testing, intestinal biopsies, and extra-intestinal symptoms at presentation, 12, 24, and greater than 24 months were recorded. Read More

    Effects of enzyme activities during steeping and sprouting on the solubility and composition of proteins, their bioactivity and relationship with the bread making quality of wheat flour.
    Food Funct 2016 Oct;7(10):4323-4331
    Institute for Multidisciplinary Research, University of Belgrade, Kneza Višeslava 1, 11030 Belgrade, Serbia.
    The aim was to determine the effect of steeping and sprouting on wheat grain proteins and the functional consequences in this regard. The solubility of proteins and the polypeptide composition of albumins, globulins, gliadins and glutenins were determined, as well as the content of non-protein nitrogen and free sulfhydryl groups (-SH), and the activity of peroxidase (POD) and lipoxygenase (LOX). In addition, the pasting viscosity of flour and protein bioactivity such as antioxidant capacity and immunoreactivity were evaluated. Read More

    Dapsone-induced pure red cell aplasia and cholestatic jaundice: A new experience for diagnosis and management.
    J Res Pharm Pract 2016 Jul-Sep;5(3):215-8
    Department of Pathology, King George Medical University, Lucknow, Uttar Pradesh, India.
    Dapsone (4,4'- diaminodiphenylsulfone) is the parent compound of the sulfones, and it has potent antiparasitic, anti-inflammatory, and immunomodulatory effects. It is used in the treatment of leprosy, dermatitis herpetiformis, and prophylactically to prevent Pneumocystis pneumonia and toxoplasmosis in patients unable to tolerate trimethoprim with sulfamethoxazole. We hereby report a case of dapsone toxicity who developed pure red cell aplasia and cholestatic jaundice in a suspected case of dermatitis herpetiformis. Read More

    Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease.
    Ann Med 2017 Feb 14;49(1):23-31. Epub 2016 Dec 14.
    b Department of Dermatology , Tampere University Hospital , Tampere , Finland.
    Dermatitis herpetiformis (DH) is an itchy blistering skin disease with predilection sites on elbows, knees, and buttocks. Diagnosis is confirmed by showing granular immunoglobulin A deposits in perilesional skin. DH is one manifestation of coeliac disease; the skin symptoms heal with gluten free diet (GFD) and relapse on gluten challenge. Read More

    [An analysis of clinical features of celiac disease patients in different ethnic].
    Zhonghua Nei Ke Za Zhi 2016 Aug;55(8):613-8
    Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
    Objective: To summarize the clinical features of different racial patients with celiac disease (CD) and analyze the disease prevalence, diagnosis and treatment in Chinese population.

    Methods: All the patients were diagnosed as CD and enrolled in Beijing United Family Hospital between January 2005 and July 2015.Clinical data including nationality, age, symptoms, endoscopic and pathological findings, outcome were collected and compared in patients from different countries. Read More

    Decreased fibrinolytic potential and morphological changes of fibrin structure in dermatitis herpetiformis.
    J Dermatol Sci 2016 Oct 6;84(1):17-23. Epub 2016 Jul 6.
    Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary. Electronic address:
    Background: Recently, high prevalence of cryofibrinogenaemia has been observed in plasma of untreated dermatitis herpetiformis (DH) patients, and the pathological IgA and TG3 deposits in the papillary dermis were found to co-localize with fibrin and fibrinogen.

    Objective: To study the fibrinolytic potential in plasma of untreated, dapsone and or/gluten-free diet treated DH patients as well as the in vitro effect of dapsone on the fibrinolytic profile.

    Method: Plasma samples of 23 DH patients, 19 healthy subjects and 5 pemphigus vulgaris patients were investigated by a turbidimetric-clot lysis assay. Read More

    Nutritional therapy - Facing the gap between coeliac disease and gluten-free food.
    Int J Food Microbiol 2016 Dec 15;239:113-124. Epub 2016 Jun 15.
    Food and Nutritional Sciences, National University Cork, College Road, Cork, Ireland. Electronic address:
    The market of gluten-free bakery products is considerably growing since better diagnostic methods allow identifying an increasing number of people suffering coeliac disease and other gluten-related disorders such as dermatitis herpetiformis, gluten ataxia, wheat allergy and non-coeliac gluten sensitivity. The only and safe treatment available nowadays for these types of disorders is to follow a strict and permanent lifelong gluten-free diet. Beside the people needing to follow a gluten-free diet for health reasons, a new segment of consumers has arisen who consume gluten-free products as a lifestyle choice. Read More

    [Celiac disease : Pathogenesis, clinics, epidemiology, diagnostics, therapy].
    Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz 2016 Jul;59(7):827-35
    Institut für Translationale Immunologie, Zentrum für Zöliakie und Dünndarmerkrankungen und Forschungszentrum für Immuntherapie (FZI), Universitätsmedizin, Johannes Gutenberg-Universität Mainz, Langenbeckstr.1, 55131, Mainz, Deutschland.
    Celiac disease is induced by the consumption of gluten containing cereals (wheat, spelt, barley, rye). With a prevalence of ~ 1 %, it is the most common non-infectious chronic inflammatory intestinal disease worldwide. It manifests in all age groups, either classically with abdominal pain, diarrhoea and growth failure or weight loss, more commonly with indirect consequences of malabsorption, such as anaemia and osteoporosis, or with associated autoimmune diseases like type 1 diabetes, autoimmune thyroiditis or dermatitis herpetiformis. Read More

    Inflammatory and glandular skin disease in pregnancy.
    Clin Dermatol 2016 May-Jun;34(3):335-43. Epub 2016 Feb 11.
    Department of Dermatology, Warren Alpert Medical School of Brown University, Providence, Rhode Island; Department of Dermatology, Rhode Island Hospital, Providence, Rhode Island.
    A switch from cell-mediated to humoral immunity (helper T 1 [Th1] to helper T 2 [Th2] shift) during gestation plays a key role in placental immune tolerance. As a result, skin diseases that are Th2 mediated often worsen, whereas skin diseases that are Th1 mediated often improve during gestation. Also, due to fluctuations in glandular activity, skin diseases involving sebaceous and eccrine glands may flare, whereas those involving apocrine glands may improve during pregnancy. Read More

    Gastrointestinal Symptoms Increase the Burden of Illness in Dermatitis Herpetiformis: A Prospective Study.
    Acta Derm Venereol 2017 Jan;96(7):58-62
    School of Medicine, University of Tampere, Tampere, Finland.
    Dermatitis herpetiformis (DH) is an extraintestinal manifestation of coeliac disease. The burden of illness in untreated coeliac disease is known to be considerable, but corresponding evidence for DH is lacking. In this study the burden of DH was evaluated prospectively in 52 patients newly diagnosed with DH using a study questionnaire and a validated Psychological General Well-Being (PGWB) questionnaire. Read More

    [Pustular psoriasis].
    Hautarzt 2016 Jun;67(6):445-53
    Klinik für Dermatologie, Venerologie und Allergologie, Universitätsmedizin Göttingen, Robert-Koch-Str. 40, UMG, 37075, Göttingen, Deutschland.
    A number of pustular skin diseases share clinical, pathogenetic, and epidemiological aspects with plaque-type psoriasis, and their classification as a separate clinical entity or as a subtype of psoriasis remains controversial, which is also reflected in the multitude of their names. They include generalized pustular psoriasis with its subtypes, acrodermatitis continua suppurativa (Hallopeau), acute pustulosis palmopantaris, palmoplantar pustular psoriasis, and pustular variants of a mostly TNF-blocker triggered paradoxical psoriasiform dermatitis. In this article, the epidemiology, clinical picture, pathogenesis, genetics, and therapy of these pustular skin diseases are described. Read More

    Cutaneous autoimmune effects in the setting of therapeutic immune checkpoint inhibition for metastatic melanoma.
    J Cutan Pathol 2016 Sep 1;43(9):787-91. Epub 2016 Jun 1.
    Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA.
    Therapeutic immune checkpoint blockade for metastatic melanoma has been associated with vitiligo, pruritus and morbilliform eruptions. Reports of other autoimmune skin disease in this setting are rare. We sought to expand the spectrum of cutaneous immune-mediated effects related to immune checkpoint inhibitor therapy. Read More

    The Decreasing Prevalence of Severe Villous Atrophy in Dermatitis Herpetiformis: A 45-Year Experience in 393 Patients.
    J Clin Gastroenterol 2017 Mar;51(3):235-239
    Departments of *Dermatology ¶Internal Medicine #Gastroenterology and Alimentary Tract Surgery, Tampere University Hospital †School of Medicine, University of Tampere, Tampere ‡Unit of Primary Health Care, Helsinki University Central Hospital §Department of General Practice, University of Helsinki, Helsinki ∥Unit of Primary Health Care, Kuopio University Hospital, Helsinki and Kuopio, Finland.
    Goals: We analyzed from our prospectively collected series of patients with dermatitis herpetiformis (DH) whether small-bowel histologic findings are changing and how serum tissue transglutaminase (TG2) IgA antibodies correlate to mucosal damage.

    Background: DH is an extraintestinal manifestation of celiac disease presenting with itchy blistering rash and pathognomonic IgA deposits in the skin. Prominent gastrointestinal symptoms are rare, and small-bowel findings range from severe villous atrophy (SVA) and partial villous atrophy (PVA) to normal mucosa with inflammatory changes. Read More

    Utility of Direct Immunofluorescence Studies in Subclassification of Autoimmune Sub-Epidermal Bullous Diseases: A 2-Year Study in a Tertiary Care Hospital.
    Turk Patoloji Derg 2016 ;32(2):91-8
    Department of Pathology Bharati Vidyapeeth Deemed University Medical College and Hospital, SANGLI, INDIA.
    Objective: Sub-epidermal bullous disorders belong to immunobullous diseases which develop as a result of autoantibody action against epidermal basement membrane proteins. Clinically, they are tense bullae and do not rupture easily. They are classified into various forms based on histopathology and direct immunofluorescence patterns. Read More

    Clinical, demographic and immunopathological spectrum of subepidermal autoimmune bullous diseases at a tertiary center: A 1-year audit.
    Indian J Dermatol Venereol Leprol 2016 May-Jun;82(3):358
    Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Background: The subepidermal autoimmune bullous diseases are a subset of immunobullous diseases encountered less frequently in the Indian population. There is a paucity of data on the prevalence, demographic and clinicopathological spectrum of various subepidermal autoimmune bullous diseases from India.

    Aim: To determine the demographic and clinicopathological profile of subepidermal autoimmune bullous diseases in Indian patients, presenting to the Immunobullous Disease Clinic of Postgraduate Institute of Medical Education and Research, Chandigarh. Read More

    Serum transglutaminase 3 antibodies correlate with age at celiac disease diagnosis.
    Dig Liver Dis 2016 Jun 9;48(6):632-7. Epub 2016 Mar 9.
    School of Medicine, University of Tampere, Tampere, Finland; Department of Internal Medicine, Tampere University Hospital, Tampere, Finland.
    Background: Transglutaminase (TG)2 is the autoantigen in celiac disease, but also TG3 antibodies have been detected in the serum of celiac disease patients.

    Aims: To investigate the correlations between serum TG3 antibodies and clinical and histological manifestations of celiac disease and to assess gluten-dependency of TG3 antibodies.

    Methods: Correlations between serum TG3 antibody levels measured from 119 adults and children with untreated coeliac disease and the demographic data, clinical symptoms, celiac antibodies, histological data and results of laboratory tests and bone mineral densities were tested. Read More

    Early Onset Dapsone-induced Photosensitive Dermatitis: A Rare Side Effect of a Common Drug.
    Indian J Lepr 2015 Jul-Sep;87(3):161-4
    Dapsone, a potent anti-inflammatory compound, is mainly used in the treatment of leprosy, dermatitis herpetiformis, erythema elevatum diutinum and other dermatoses. Cutaneous adverse reactions range from acneiform eruptions to toxic epidermal necrolysis. A 30-year-old, married women who was treated with paucibacillary multi drug therapy, developed itchy skin lesions over the both forearms, 'V ' area of the neck and upper back after one week of the drug administration which worsened on exposure to sunlights. Read More

    Extraintestinal manifestations of celiac disease: 33-mer gliadin binding to glutamate receptor GRINA as a new explanation.
    Bioessays 2016 May 18;38(5):427-39. Epub 2016 Mar 18.
    Department of Biochemistry and Molecular Biology, School of Pharmacy, University of Seville, Spain.
    We propose a biochemical mechanism for celiac disease and non-celiac gluten sensitivity that may rationalize many of the extradigestive disorders not explained by the current immunogenetic model. Our hypothesis is based on the homology between the 33-mer gliadin peptide and a component of the NMDA glutamate receptor ion channel - the human GRINA protein - using BLASTP software. Based on this homology the 33-mer may act as a natural antagonist interfering with the normal interactions of GRINA and its partners. Read More

    The HLA Alleles B*0801 and DRB1*0301 Are Associated with Dermatitis Herpetiformis in a Chinese Population.
    J Invest Dermatol 2016 Feb 18;136(2):530-2. Epub 2015 Nov 18.
    Shandong Provincial Institute of Dermatology and Venereology, Shandong Academy of Medical Sciences, Jinan, Shandong, China; Shandong Provincial Hospital for Skin Diseases, Shandong University, Jinan, Shandong, China; Shandong Provincial Key Lab for Dermatovenereology, Jinan, Shandong, China; School of Medicine, Shandong University, Jinan, Shandong, China; National Clinical Key Project of Dermatology and Venereology, Jinan, Shandong, China. Electronic address:

    A lethal case of the dapsone hypersensitivity syndrome involving the myocardium.
    Neth J Med 2016 Feb;74(2):89-92
    Department of Infectious disease, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
    In the Netherlands dapsone is used for the treatment of dermatitis herpetiformis, leprosy and Pneumocystis jiroveci pneumonia and prophylaxis in case of cotrimoxazole allergy. An idiosyncratic drug reaction, known as the dapsone hypersensitivity syndrome (DHS), appears in about 0.5-3. Read More

    The frequency of mucosal-associated invariant T cells is selectively increased in dermatitis herpetiformis.
    Australas J Dermatol 2016 Mar 4. Epub 2016 Mar 4.
    Department of Microbiology and Immunology, Peter Doherty Institute for Infection and Immunity, University of Melbourne, Melbourne, Victoria, Australia.
    Background/objectives: Mucosal-associated invariant T (MAIT) cells are a novel subset of innate-like T-cells that are enriched in mucosal tissues. Their presence in human skin has only recently been recognised. We describe the expression of skin-tropic molecules on human skin MAIT cells at steady state and investigate their contribution to various dermatoses with known T-cell involvement. Read More

    Acute Poisoning with Dapsone and Olanzapine: Severe Methemoglobinemia and Coma with a Favourable Outcome.
    Folia Med (Plovdiv) 2015 Apr-Jun;57(2):122-6
    Dapsone is a drug commonly used in the treatment of leprosy. In Europe it is rarely prescribed, mostly for the treatment of skin diseases such as dermatitis herpetiformis. Poisoning with dapsone is rare and reports of such cases are of interest for toxicological practice. Read More

    Granular C3 Dermatosis.
    Acta Derm Venereol 2016 Aug;96(6):748-53
    Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 830-0011 Kurume, Japan.
    There has been no previous systematic study of bullous skin diseases with granular basement membrane zone deposition exclusively of C3. In this study we collected 20 such patients, none of whom showed cutaneous vasculitis histopathologically. Oral dapsone and topical steroids were effective. Read More

    Prospects of Developing Medicinal Therapeutic Strategies and Pharmaceutical Design for Effective Gluten Intolerance Treatment.
    Curr Pharm Des 2016 ;22(16):2439-49
    Sechenov First Moscow State Medical University, Institute of Molecular Medicine, Trubetskaya str. 8, bld. 2, Moscow, 119991, Russia.
    Gluten intolerance is an umbrella term for gluten-related disorders manifested in health decline as a result of the gluten ingestion. The spectrum of gluten-related disorders includes three major groups: autoimmune (mainly, Celiac Disease, CD, also known as Celiac Sprue, dermatitis herpetiformis, or gluten-sensitive ataxia), allergic (wheat allergy, WA), and non-autoimmune non-allergic (non-celiac gluten sensitivity, NCGS, or gluten sensitivity, GS). Pathogenesis and diagnostics of CD and WA are well established in contrast to NCGS, pathogenicity of which is still poorly understood and its symptoms are frequently misdiagnosed since most of the NCGS cases are currently identified via the process of CD and WA exclusion. Read More

    Spectrum of autoimmune vesiculobullous diseases in Iran: a 13-year retrospective study.
    Clin Cosmet Investig Dermatol 2016 11;9:15-20. Epub 2016 Jan 11.
    Psoriasis Research Center, Department of Dermatology, Farshchian Hospital, Hamadan University of Medical Sciences, Hamadan, Iran.
    Background: Autoimmune bullous diseases (ABDs) represent a group of rare, acquired disorders characterized by overlapping features with involvement of the skin and mucous membranes, resistance to treatment, and potential lethality that comprise pemphigus, bullous pemphigoid (BP), epidermolysis bullosa, dermatitis herpetiformis, and linear immunoglobulin A bullous dermatosis.

    Aim: The main aim of this study was to identify the epidemiologic characteristics and clinical courses of these common diseases in Hamadan, Iran. Few surveys have been carried out to demonstrate the whole spectrum of ABDs in the literature. Read More

    Literature-based immunization recommendations for patients requiring immunosuppressive medications for autoimmune bullous dermatoses.
    Int J Dermatol 2016 Jun 29;55(6):599-607. Epub 2015 Dec 29.
    Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
    Autoimmune bullous diseases, such as pemphigus, pemphigoid, and dermatitis herpetiformis, are uniquely associated with vulnerability in the mucocutaneous barrier against infection. The management of immunobullous diseases is complex and may at times require immunosuppressive medications. Iatrogenic immunosuppression may increase susceptibility to vaccine-preventable illnesses. Read More

    Bullous diseases: Kids are not just little people.
    Clin Dermatol 2015 Nov-Dec;33(6):644-56. Epub 2015 Sep 14.
    Departments of Dermatology & Pediatrics, Columbia University College of Physicians and Surgeons, 161 Fort Washington Avenue, 12th Floor, New York, NY, 10032, USA. Electronic address:
    Bullous diseases may be rare; however, this does not preclude the clinician from being familiar with their manifestations and treatment. After ruling out infection, genetically inherited blistering diseases are more likely to be the cause of blistering or erosions in the neonatal period, whereas immunobullous diseases are more common in adults. Published literature on immunobullous disorders reflects information gleaned from case reports and open-label case series; prospective studies and evidence-based treatments are limited. Read More

    Dermatitis Herpetiformis: A Review of Direct Immunofluorescence Findings.
    Am J Dermatopathol 2016 Apr;38(4):283-8
    Departments of *Dermatology, and †Pathology, Hospital Sta. Creu I St. Pau, Barcelona, Spain.
    Direct immunofluorescence (DIF) findings in dermatitis herpetiformis (DH) are incompletely defined. The presence and localization of immune reactants in this disorder are reviewed. A retrospective study on 72 biopsies from 71 patients with DH was performed. Read More

    Utility of direct immunofluorescence testing for IgA in patients with high and low clinical suspicion for dermatitis herpetiformis.
    Am J Clin Pathol 2015 Dec;144(6):880-4
    From the Department of Pathology, Brigham and Women's Hospital, and Harvard Medical School, Boston, MA.
    Objectives: The purpose of this study was to examine the utility of direct immunofluorescence (DIF) testing for the characteristic immunoglobulin A deposits of dermatitis herpetiformis (DH) in patients stratified into high and low clinical suspicion subgroups.

    Methods: We retrospectively analyzed the results of H&E and DIF testing in 77 cases of suspected DH and separated them into high and low clinical suspicion subgroups based on clinical impression at the time of biopsy.

    Results: The overall sensitivity and specificity of routine (H&E) histologic evaluation were 0. Read More

    Celiac Disease: Diagnosis.
    Methods Mol Biol 2015 ;1326:15-22
    Immunology Department, Trinity College, Dublin, Ireland.
    Historically the diagnosis of celiac disease has relied upon clinical, serological, and histological evidence. In recent years the use of sensitive serological methods has meant an increase in the diagnosis of celiac disease. The heterogeneous nature of the disorder presents a challenge in the study and diagnosis of the disease with patients varying from subclinical or latent disease to patients with overt symptoms. Read More

    Symptoms and findings in adult-onset celiac disease in a historical Danish patient cohort.
    Scand J Gastroenterol 2016 Mar 9;51(3):288-94. Epub 2015 Oct 9.
    a Department of Hepatology and Gastroenterology , Aarhus University Hospital , Aarhus C , Denmark.
    Objective: The presentation of celiac disease (CD) has changed over the past decades. We aimed to describe the incidence of CD and its complications at diagnosis in a historical cohort in a well-defined population in Denmark.

    Methods: We included all patients aged 15+ years, who lived in Aarhus County, Denmark, and who were diagnosed with CD between January 2008 and August 2013. Read More

    [Expression of transient receptor potential lvanilloidreceptor 4 protein in autoimmune bullous skin disorders].
    Nan Fang Yi Ke Da Xue Xue Bao 2015 Aug;35(9):1349-51
    Department of Dermatology, Second Affiliated Hospital, Xi'an Jiaotong University College of Medicine, Xi'an 710004, China.E-mail:
    Objevtive: To investigate the expression of transient receptor potential lvanilloidreceptor 4 (TRPV4) protein in pemphigus vulgaris (PV), bullous pemphigoid (BP), dermatitis herpetiformis (DH), and epidermolysis bullosa acquisita (EBA), and explore the role of TRPV4 in the pathogenesis of these diseases.

    Methods: TRPV4 protein in normal skin tissues and lesions of PV, BP, DH, and EBA were detected with immunohistochemistry.

    Results: The positivity rate of TRPV4 protein expression was 61. Read More

    Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients.
    Pediatr Dermatol 2015 Nov-Dec;32(6):845-52. Epub 2015 Sep 22.
    Division of Dermatology, National University Hospital, Singapore, Singapore.
    Background: Autoimmune blistering diseases (AIBDs) are rare in children and their prevalence in Singapore is unclear. We aimed to investigate the clinical and immunopathologic characteristics of children diagnosed with AIBDs in Singapore.

    Materials And Methods: The clinical and histology databases at the National Skin Centre in Singapore were searched to identify patients younger than 18 years old diagnosed with an AIBD from January 1, 1998, through December 31, 2012. Read More

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