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https://www.readbyqxmd.com/read/28079922/iga-tracheobronchial-deposits-underlie-respiratory-compromise-in-neonatal-linear-iga-bullous-dermatosis
#1
A Diociaiuti, G Zambruno, F Diomedi Camassei, G Di Zenzo, I Capolupo, F Stoppa, V Forziati, M El Hachem
The childhood variant of linear IgA bullous dermatosis (LABD) is characterized by blistering lesions of the perineum and perioral area with an annular or "rosette-like" arrangement, in addition to the pathognomonic linear IgA deposits along the cutaneous basement membrane zone (BMZ).(1) The disease mainly occurs in preschool children.(1) However, a handful of neonatal cases have been described, most of them presenting with severe mucosal involvement.(2-8) We report an additional case of neonatal LABD with life-threatening respiratory tract manifestations...
January 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28079339/survey-of-bullous-pemphigoid-in-an-italian-university-hospital-clinical-epidemiological-characteristics-and-follow-up
#2
Riccardo Balestri, Giulia Odorici, Annalisa Patrizi, Salvatore D Infusino, Michela Magnano, Federico Bardazzi
BACKGROUND: The clinical-epidemiological characteristics and course of bullous pemphigoid in the general population is not clear. Few studies have been performed to date, and only one in the Italian population more than ten years ago. We decided to evaluate the characteristics and outcome of patients admitted for a bullous pemphigoid at our Hospital in the last 4 years. METHODS: We retrospectively review the last 4 years' medical records of the Department of Dermatology of the University of Bologna, identifying all patients with histological and immunological data typical for bullous pemphigoid...
January 12, 2017: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/28052410/can-immunohistochemistry-replace-immunofluorescence-in-diagnosis-of-skin-bullous-diseases
#3
Hanan Al-Saeid Al-Shenawy
Autoimmune bullous diseases are distressing and sometimes risky bullous dermatoses characterized by the presence of antibodies focused against disease-specific target antigens. Recognition of these antibodies using immunofluorescence is used to be the only sure diagnostic method after reviewing the routine histopathological section. Because of many causes that make the using of immunofluorescence difficult, we tried to evaluate the role of immunohistochemistry in diagnosis of these bullous skin diseases; 40 pemphigus cases (30 pemphigus vulgaris and 10 pemphigus foliaceus) and 37 non-pemphigus cases (35 vesiculobullous skin diseases and 2 normal skin)...
January 3, 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28038889/periodontitis-in-oral-pemphigus-and-pemphigoid-a-systematic-review-of-published-studies
#4
REVIEW
Isabella Jascholt, Olivia Lai, Detlef Zillikens, Michael Kasperkiewicz
Periodontitis and autoimmune bullous diseases, including pemphigus vulgaris and mucous membrane pemphigoid, are immunoinflammatory disorders leading to microbial plaque- and autoantibody-elicited tissue injury of the oral cavity, respectively. Evidence indicates that these autoimmune conditions may represent a risk factor for periodontitis, but no systematic evaluation exists to corroborate this assumption. A systematic literature review of periodontal status in pemphigus and pemphigoid was conducted. Electronic searches using PubMed from inception to July 2016 identified 10 studies meeting predetermined inclusion and exclusion criteria...
December 28, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28038887/prospective-studies-on-the-routine-use-of-a-novel-multivariant-enzyme-linked-immunosorbent-assay-for-the-diagnosis-of-autoimmune-bullous-diseases
#5
Nina van Beek, Cornelia Dähnrich, Nora Johannsen, Susanne Lemcke, Stephanie Goletz, Franziska Hübner, Giovanni Di Zenzo, Marian Dmochowski, Kossara Drenovska, Shamir Geller, Michael Horn, Cezary Kowalewski, Ljiljana Medenica, Dedee F Murrell, Aikaterini Patsatsi, Soner Uzun, Snejina Vassileva, Detlef Zillikens, Wolfgang Schlumberger, Enno Schmidt
BACKGROUND: Serologic diagnosis of autoimmune blistering disease (AIBD) usually follows a sophisticated multistep algorithm. OBJECTIVE: We sought validation of a multivariant enzyme-linked immunosorbent assay (ELISA) in the routine diagnosis of AIBD. METHODS: The multivariant ELISA comprising 6 recombinant immunodominant forms of major AIBD target antigens, ie, desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, and type VII collagen was applied in: (1) a cohort of well-characterized AIBD (n = 173) and control sera (n = 130), (2) a prospective multicenter study with 204 sera from patients with newly diagnosed AIBD with positive direct immunofluorescence microscopy, and (3) a prospective monocenter study with 292 consecutive sera from patients with clinical suspicion of AIBD in comparison with the conventional multistep diagnostic algorithm...
December 28, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28012821/validation-of-chemiluminescent-enzyme-immunoassay-in-detection-of-autoantibodies-in-pemphigus-and-pemphigoid
#6
Yumi Fujio, Kazuo Kojima, Masahiro Hashiguchi, Masatoshi Wakui, Mitsuru Murata, Masayuki Amagai, Jun Yamagami
BACKGROUND: A novel chemiluminescent enzyme immunoassay (CLEIA) was recently developed to quantify autoantibodies specific for desmogleins (Dsgs) and BP180, the target antigens of pemphigus and pemphigoid. This assay is automated and highly accurate and efficient. OBJECTIVE: To validate the use of the CLEIA for detection of autoantibodies during the clinical courses of patients with pemphigus and pemphigoid. METHODS: To define cut-off values for Dsg1, Dsg3, and BP180, we evaluated 47 serum samples from patients with pemphigus foliaceus (PF), 59 from those with pemphigus vulgaris (PV), 52 from those with bullous pemphigoid (BP), and 995 from healthy individuals...
December 6, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28011091/-oral-care-recommendations-for-patients-with-oral-autoimmune-bullous-diseases
#7
V Sobocinski, S-M Dridi, C Bisson, S Jeanne, F Gaultier, C Prost-Squarcioni, P Bernard, F Pascal, B Lefevre, P Weber, C Abasq, S Agbo-Godeau, P Joly, S Ingen-Housz-Oro, S Duvert-Lehembre
BACKGROUND: Autoimmune bullous diseases (AIBD) may cause chronic oral lesions that progress insidiously. AIMS: To provide recommendations for optimal oral-dental management of patients presenting AIBD with oral involvement. PATIENTS AND METHODS: In the absence of scientific studies with high levels of proof, these recommendations have been drawn up at two meetings by a committee of experts on AIBD comprising 7 dermatologists, 1 stomatologist, 1 maxillofacial surgeon, 2 odontologists and 4 parodontologists...
December 20, 2016: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28010761/research-techniques-made-simple-mouse-models-of%C3%A2-autoimmune-blistering-diseases
#8
Robert Pollmann, Rüdiger Eming
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...
January 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28009404/vogt-koyanagi-harada-syndrome-uveomeningoencephalitic-syndrome
#9
Katerina Manethova, Jan Ernest, Michal Hrevus
PURPOSE: To report a case of a 29-year-old man who was examined at the Eye Clinic of Central Military University Hospital Prague for a severe headache and acute blurring of vision in both eyes diagnosed as incomplete Vogt-Koyanagi-Harada syndrome (VKH). METHODS: This is a retrospective and descriptive case report based on data from clinical records, patient observation and follow-ups and analysis of acquired diagnostic tests. RESULTS: A 29-year-old man presented with headache and decreased vision in his left eye (LE) for 2 days...
November 25, 2016: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/28003921/bullous-dermatosis-in-an-end-stage-renal-disease-patient-a-case-report-and-literature-review
#10
Zeenat Yousuf Bhat, Marwan Abu Minshar, Nashat Imran, Andrew Thompson, Yahya Osman Malik
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...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27995774/occult-primary-pulmonary-synovial-sarcoma-presenting-as-recurrent-spontaneous-pneumothorax-and-explosive-progression
#11
Chenglin Guo, Chengwu Liu, Qiang Pu, Feng Lin, Lunxu Liu
Primary pulmonary synovial sarcoma (PPSS) is a relatively rare neoplasm with highly progressive potential. We present an extremely rare case of PPSS presenting as recurrent pneumothorax with bullous lesions. Bullectomy was performed at the local hospital. Unfortunately, the patient was initially misdiagnosed as atypical carcinoid. Although a negative resection margin was obtained during the first surgery and a remedial operation and chemotherapy followed, the patient developed severe disease progression and died soon after...
December 20, 2016: Thoracic Cancer
https://www.readbyqxmd.com/read/27995619/idiopathic-linear-iga-bullous-dermatosis-prognostic-factors-based-on-a-case-series-of-72-adults
#12
J Gottlieb, S Ingen-Housz-Oro, M Alexandre, S Grootenboer-Mignot, F Aucouturier, E Sbidian, E Tancrede, P Schneider, E Regnier, C Picard-Dahan, E Begon, C Pauwels, K Cury, S Hüe, C Bernardeschi, N Ortonne, F Caux, P Wolkenstein, O Chosidow, C Prost-Squarcioni
BACKGROUND: Linear IgA bullous dermatosis (LABD) is a clinically and immunologically heterogeneous, subepidermal, autoimmune blistering disease (AIBD), whose long-term evolution is poorly described. OBJECTIVES: To investigate the clinical and immunological characteristics, follow-up and prognostic factors of adult idiopathic LABD. METHODS: This retrospective study, conducted in our AIBD Referral Center, included adults, diagnosed between 1995 and 2012, with idiopathic LABD, defined as pure or predominant IgA deposits by direct immunofluorescence...
December 20, 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/27974963/bullous-systemic-lupus-erythematosus-and-lupus-nephritis-in-a-young-girl
#13
Tooba Momen, Yahya Madihi
Bullous systemic lupus erythematosus (BSLE) is an autoimmune blistering disease occurring in patients with systemic lupus erythematosus (SLE). It is a rare disease, especially in children. A 14-year-old girl initially presented with fatigue, generalized vesiculobullous skin lesions, and ulcers over the hard palate and oral mucosa. Clinical investigations revealed hematuria and proteinuria, a high erythrocyte sedimentation rate and titer of antinuclear antibody, and anti-double-stranded DNA. Skin biopsy findings were suggestive of BSLE...
November 2016: Oman Medical Journal
https://www.readbyqxmd.com/read/27957424/bullous-skin-lesions-in-a-patient-with-end-stage-renal-disease-combined-with-myeloma-and-primary-amyloidosis
#14
Jong Hwan Jung
No abstract text is available yet for this article.
December 2016: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/27943257/a-clinical-and-serological-study-of-linear-iga-bullous-dermatosis-without-linear-immunoglobulin-deposition-other-than-iga-at-the-basement-membrane-zone-in-direct-immunofluorescence
#15
C Ohata, N Ishii, H Koga, T Nakama
BACKGROUND: Linear IgA bullous dermatosis (LABD) is a heterogeneous disease. Different diagnostic criteria have been used in different reports. OBJECTIVES: We aimed to reappraise the characteristic features of LABD with only IgA deposition at the basement membrane zone (BMZ) in direct immunofluorescence (DIF). METHODS: We retrospectively collected 101 patients who had a) blisters on the skin and/or mucous membrane, b) subepidermal blisters in a biopsy specimen, and c) linear IgA deposition along BMZ with/without linear C3 deposition at the BMZ in DIF from our archival records from January 1, 1996, through December 31, 2014...
December 11, 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/27914107/non-paraneoplastic-autoimmune-subepidermal-bullous-disease-associated-with-fatal-bronchiolitis-obliterans
#16
Mari Orime, Katsuhiro Tomiyama, Hideki Hashidate, Satoru Yoshida, Satoshi Hokari, Akiko Tsuda, Hisashi Yokoyama, Jun-Ichi Narita, Youhei Uchida, Takuro Kanekura, Riichiro Abe, Norito Ishii, Takashi Hashimoto, Kazuhiro Kawai
Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation...
December 3, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27899204/stevens-johnson-syndrome-in-childhood
#17
N Blanco, B Gutiérrez, I Valls, D Puertas, C Martín, M Rivera, Á Hernández, A Torrelo
INTRODUCTION: Stevens-Johnson syndrome and toxic epidermal necrolysis are two serious immune diseases within the context of bullous mucocutaneous syndrome. These have varying degrees of involvement of the skin and usually at least two mucous membranes. CLINICAL CASES: Three clinical cases are presented, two of them with significant ophthalmological sequelae, who had received drug treatment as a possible trigger, and another milder clinical case caused by Mycoplasma pneumoniae...
November 26, 2016: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/27893467/bullous-pyoderma-gangrenosum-with-subungual-involvement-associated-with-ulcerative-colitis
#18
Ezgi Aktaş Karabay, Asl Aksu Cerman, İlknur Kvanc Altunay, Özben Yalçn
Pyoderma gangrenosum (PG) is a rare inflammatory and ulcerative skin disease of unknown etiology characterized by neutrophilic infiltration of the dermis, mainly affecting the lower extremities. Bullous PG is a rare variant of this disease, usually associated with hematologic disorders. Here, we report a case of pathergy-positive bullous PG with subungual involvement associated with ulcerative colitis.
November 22, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27885526/twelve-year-follow-up-of-penetrating-keratoplasty
#19
Takashi Ono, Sosuke Ishiyama, Takeshi Hayashidera, Yosai Mori, Ryohei Nejima, Kazunori Miyata, Shiro Amano
PURPOSE: To evaluate the long-term outcomes of penetrating keratoplasty (PKP) according to the corneal disease diagnosis and the number of PKP procedures performed. METHODS: Five-hundred-and-nine eyes from 403 patients who underwent PKP at Miyata Eye Hospital in Japan from 1998 through 2014, were included in this study. Medical charts were retrospectively examined to ascertain the corneal disease diagnosis and the period of graft survival. Graft survival rates were compared among various corneal disease diagnoses and among the number of PKP procedures performed...
November 24, 2016: Japanese Journal of Ophthalmology
https://www.readbyqxmd.com/read/27878477/paraneoplastic-pemphigus-and-autoimmune-blistering-diseases-associated-with-neoplasm-characteristics-diagnosis-associated-neoplasms-proposed-pathogenesis-treatment
#20
REVIEW
Saritha Kartan, Vivian Y Shi, Ashley K Clark, Lawrence S Chan
Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Autoimmune paraneoplastic and neoplasm-associated bullous skin syndromes are characterized by blister formation due to an autoimmune response to components of the epidermis or basement membrane in the context of a neoplasm...
November 22, 2016: American Journal of Clinical Dermatology
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