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Autoimmune bullous child

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https://www.readbyqxmd.com/read/25593817/childhood-lichen-planus-pemphigoides-triggered-by-chickenpox
#1
T S Mohanarao, Gummalla Ajay Kumar, Kavya Chennamsetty, T Priyadarshini
Lichen planus pemphigoides (LPP) is a rare autoimmune bullous disorder that is rarer in children characterized by bullae on lichen planus like papules and the normal looking skin. Clinical, histopathological and direct immunoflourescence evaluation is important for the diagnosis of this entity. We report a case of LPP in a 5-year-old girl child probably triggered by an episode of preceding varicella. Diagnosis was confirmed by histopathology and immunofluorescence examination. Histological findings were typical of lichen planus from the biopsy taken from a violaceous to erythematous papule and characteristic of bullous pemphigoid in the biopsy taken from a bullous lesion with evidence of immunoglobulin G and C3 deposition along the basement membrane zone on direct immunofluorescence...
December 2014: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/24372990/clinical-predictors-of-autoimmune-and-severe-atopic-disease-in-pediatric-heart-transplant-recipients
#2
Jessica H Mouledoux, Erin L Albers, Zengqi Lu, Benjamin R Saville, Daniel J Moore, Debra A Dodd
Autoimmune and allergic diseases cause morbidity and diminished quality of life in pediatric organ transplant recipients. We hypothesize that younger age at transplantation and immunosuppression regimen play a role in the development of immune-mediated disease following heart transplant. A single institution retrospective review identified all patients undergoing heart transplant at ≤18 yr of age from 1987 to 2010 who survived ≥1 yr. Using medical record and database review, patients were evaluated for development of autoimmune or severe allergic disease...
March 2014: Pediatric Transplantation
https://www.readbyqxmd.com/read/24346883/linear-iga-bullous-dermatosis-report-of-an-exuberant-case
#3
Beatriz Cavalcanti de Souza, Nádire Cristina Freire Pontes Fregonesi, Antônio José Tebcherani, Ana Paula Galli Sanchez, Valéria Aoki, Juliana Christien Fernandes
Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.
November 2013: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/23951793/characterising-oral-lesions-in-patients-who-presented-with-chronic-vesiculobullous-lesions-during-a-10-year-period
#4
N H Wood, S L Shangase
INTRODUCTION: Vesiculobullous lesions are a subset of lesons with an aetiologic spectrum that ranges from infection to aberrant mmune function. The bulk are chronic immune-mediated or autoimmune diseases in which inappropriate production of antibodies against auto-antigens results in the development of these conditions. METHODS: All departmenta patient records in the ten-year period were reviewed and those indicating a provisional diagnosis of oral chronic immune-mediated lesions were included in the study...
August 2012: SADJ: Journal of the South African Dental Association
https://www.readbyqxmd.com/read/23433193/post-surgical-visual-outcome-and-complications-in-boston-type-1-keratoprosthesis
#5
G Muñoz-Gutierrez, J Alvarez de Toledo, R I Barraquer, L Vera, R Couto Valeria, J Nadal, M F de la Paz
OBJECTIVE: To describe the visual outcome of patients who underwent Boston type 1 keratoprosthesis (KPro1) implantation, and describe serious sight-threatening post-operative complications. METHODS: We performed an analysis of the clinical records of all patients who underwent Boston keratoprosthesis implantation (BKI)in our institution from May 2006 to February 2011. RESULTS: A total of 41 eyes of 37 patients were included in the final analysis, of whom 22 (59...
February 2013: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/23426192/prevalence-and-clinical-significance-of-anti-laminin-332-autoantibodies-detected-by-a-novel-enzyme-linked-immunosorbent-assay-in-mucous-membrane-pemphigoid
#6
MULTICENTER STUDY
Philippe Bernard, Frank Antonicelli, Christophe Bedane, Pascal Joly, Christelle Le Roux-Villet, Sophie Duvert-Lehembre, Patricia Rousselle, Catherine Prost-Squarcioni
IMPORTANCE: A rare variant of mucous membrane pemphigoid (MMP) is characterized by circulating anti-laminin 332 (Lam332) autoantibodies and seems to be associated with concurrent malignant neoplasms. OBJECTIVE: To determine the prevalence and clinical significance of anti-Lam332 autoantibody detection from a large series of patients with MMP. DESIGN: Multicenter retrospective study. SETTING: Four French national centers for autoimmune bullous diseases...
May 2013: JAMA Dermatology
https://www.readbyqxmd.com/read/23252883/detection-of-igg-and-ige-reactivity-to-bp180-using-the-isac%C3%A2-microarray-system
#7
D Pomponi, G Di Zenzo, D Zennaro, V Calabresi, R Eming, S Zuzzi, M L Bernardi, E Scala, A Mari
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune skin disease in which patient autoantibodies react with BP180 and BP230 proteins. In addition to IgG, IgE has been shown to play a role in the disease. OBJECTIVES: To evaluate the feasibility of detecting IgE and IgG against the immunodominant BP180 NC16A domain (BP180) using a microarray system. METHODS: BP180 was immobilized on an experimental version of the ISAC(®) microarray (Exp96)...
June 2013: British Journal of Dermatology
https://www.readbyqxmd.com/read/23075641/evaluation-of-the-combination-of-bp180-nc16a-enzyme-linked-immunosorbent-assay-and-bp230-enzyme-linked-immunosorbent-assay-in-the-diagnosis-of-bullous-pemphigoid
#8
Baoqi Yang, Chong Wang, Shengli Chen, Xuechao Chen, Xianmei Lu, Hongqing Tian, Meiling Yu, Dizhan Zhang, Zhongxiang Shi, Guizhi Zhou, Furen Zhang
BACKGROUND: Bullous pemphigoid (BP) is an acquired autoimmune subepidermal blistering disease characterized by circulating IgG autoantibodies directed against BP180 and BP230 hemidesmosomal proteins. Previous studies have demonstrated that antibodies against the NC16a domain of BP180 mediate BP pathogenesis, while antibodies against BP230 enhance the inflammatory response. Recently, commercial BP180-NC16a enzyme-linked immunosorbent assay (ELISA) and BP230 ELISA kits were developed to detect anti-BP180 and anti-BP230 autoantibodies in human BP sera...
November 2012: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/23067077/linear-iga-bullous-dermatosis-report-of-five-cases-in-chile
#9
Mauricio Sandoval, María Magdalena Farias, Sergio Gonzalez
BACKGROUND: Linear IgA bullous dermatosis (LABD) is an acquired autoimmune sub-epidermal vesiculobullous disease characterized by continuous linear IgA deposit on the basement membrane zone, as visualized on direct immunofluorescence microscopy. LABD can affect both adults and children. The disease is very uncommon, with a still unknown incidence in the South American population. MATERIALS AND METHODS: All confirmed cases of LABD by histological and immunofluorescence in our hospital were studied...
November 2012: International Journal of Dermatology
https://www.readbyqxmd.com/read/22960821/childhood-pemphigus-vulgaris-successfully-treated-with-rituximab
#10
Amrinder J Kanwar, Gitesh U Sawatkar, Keshavamurthy Vinay, Takashi Hashimoto
Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Rituximab is a novel therapy for the treatment of refractory pemphigus. However, there is limited clinical data on safety and efficacy of rituximab in pediatric age group. Herein, we report an 11-year-old boy of childhood pemphigus vulgaris who failed to respond to dexamethasone pulse therapy and was subsequently treated with rituximab and achieved complete remission.
September 2012: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/22924407/fluorescence-overlay-antigen-mapping-using-laser-scanning-confocal-microscopy-differentiates-linear-iga-bullous-dermatosis-from-epidermolysis-bullosa-acquisita-mediated-by-iga
#11
K Wozniak, T Hashimoto, N Ishii, H Koga, M Huczek, C Kowalewski
BACKGROUND: Linear IgA bullous dermatosis (LABD) and epidermolysis bullosa acquisita (EBA) mediated by IgA antibodies belong to the group of autoimmune subepidermal bullous diseases mediated by IgA autoantibodies. Early and correct diagnosis is crucial because the management and prognosis of the diseases are different. OBJECTIVES: To determine whether fluorescence overlay antigen mapping using laser scanning confocal microscopy (FOAM-LSCM) is helpful in the differentiation between these diseases...
March 2013: British Journal of Dermatology
https://www.readbyqxmd.com/read/22837563/chronic-bullous-disease-of-childhood-with-igg-predominance-what-is-the-locus-standi
#12
Nayeem Sadath Haneef, S Ramachandra, Arun Kumar Metta, L Srujana
Linear IgA disease (LAD) is an acquired, autoimmune, subepidermal, blistering disease, characterized by linear deposition of IgA along the dermoepidermal junction on immunofluorescence. Some cases known as 'mixed immunobullous disease' show weak staining with other immune reactants like IgG, IgM or C3. We report a rare case of a child having typical manifestations of LAD (chronic bullous disease of childhood), but with IgG predominance rather than IgA. Obviously it is improper to term this as linear IgA disease...
July 2012: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/22826496/profile-of-trypanosoma-cruzi-reactivity-in-a-population-at-high-risk-for-endemic-pemphigus-foliaceus-fogo-selvagem
#13
Joaquim X Sousa, Luis A Diaz, Donald P Eaton, Günter Hans-Filho, Elder Lanzani de Freitas, Livia Delgado, Ligia Maria F Ichimura, Flávia Cristaldi, Renata Orlandi, Norival Kesper, Eufrosina S Umezawa, Evandro A Rivitti, Valeria Aoki
Fogo selvagem (FS) is an autoimmune bullous disease with pathogenic IgG autoantibodies recognizing desmoglein 1 (Dsg1), a desmosomal glycoprotein. In certain settlements of Brazil, a high prevalence of FS (3%) is reported, suggesting environmental factors as triggers of the autoimmune response. Healthy individuals from endemic areas recognize nonpathogenic epitopes of Dsg1, and exposure to hematophagous insects is a risk factor for FS. Fogo selvagem and Chagas disease share some geographic sites, and anti-Dsg1 has been detected in Chagas patients...
October 2012: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/22439305/bullous-systemic-lupus-erythematosus-with-lupus-nephritis-a-rare-case-of-a-subepidermal-bullous-disorder-in-a-child
#14
Shital Poojary, Sama Rais
Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus (SLE) with bullous lesions in a case fulfilling the American Rheumatism Association (Atlanta) criteria, histologically characterized by a neutrophil-predominant infiltrate in the upper dermis with immunoglobulin (IgG, IgA, IgM) and C3 deposition at the basement membrane zone (BMZ). It often is associated with autoimmunity to type VII collagen (NC1 [noncollagenous domain 1] domain), although occasionally other antigens such as laminin 5, laminin 6, and BP230 (bullous pemphigoid antigen) have been described...
January 2012: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/22207426/evaluation-of-clinical-and-histopathologic-direct-immunofluorescence-diagnosis-in-autoimmune-vesiculobullous-dermatitis-utility-of-direct-immunofluorescence
#15
Banu Lebe, Gülen Gül Nıflıoğlu, Selen Seyrek, Hülya Ellıdokuz
OBJECTIVE: Autoimmune bullous diseases are heterogeneous diseases and the differentiation between the various bullous disease is important for treatment and prognosis. Direct immunofluorescence microscopy is still the gold standard in differentiating these diseases. Our aim was to determine the diagnostic accordance between clinical and histopathological/direct immunofluorescence diagnosis of patients with autoimmune vesicolulobullous skin diseases. MATERIAL AND METHOD: A total of 197 cases with clinical diagnosis of vesiculobullous dermatitis was included in the study...
2012: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/22176540/efficacy-and-safety-of-rituximab-treatment-in-indian-pemphigus-patients
#16
A J Kanwar, D Tsuruta, K Vinay, H Koga, N Ishii, T Dainichi, T Hashimoto
BACKGROUND: Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Various treatment modalities have been described to treat pemphigus. In cases where the disease fails to respond to conventional therapy, rituximab has been shown to be effective. OBJECTIVE: To study the efficacy of rituximab in the treatment of resistant or severe pemphigus in Indian patients. METHODS: Patients with pemphigus were treated with intravenous rituximab 1000 mg in adults or 375 mg/m(2) body surface area in children by two doses, 15 days apart in this open labelled pilot study...
January 2013: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/22137235/autoimmune-bullous-diseases-in-childhood
#17
REVIEW
Freda Sansaricq, Sarah L Stein, Vesna Petronic-Rosic
Autoimmune blistering disorders are a heterogeneous group of diseases that result from autoantibodies generated against target antigens found in the skin and mucous membranes. This process leads to a variety of disruptions in keratinocyte adhesion and cellular integrity, resulting in fluid accumulation and development of blisters. Physicians should have an appreciation and understanding of autoimmune blistering disorders in the pediatric population when formulating a differential diagnosis of a patient who presents with skin blistering...
January 2012: Clinics in Dermatology
https://www.readbyqxmd.com/read/22137226/pemphigoid-gestationis
#18
REVIEW
Jasna Lipozenčić, Suzana Ljubojevic, Zrinka Bukvić-Mokos
Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis that occurs during pregnancy and postpartum. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of complement along the basement membrane zone of perilesional skin. The disorder is accompanied by severe pruritus and polymorphous bullous skin lesions. Clinical diagnosis is confirmed by histology and positive cutaneous immunofluorescence and immunoelectron microscopy tests (linear deposition of C3, with or without immunoglobulin G, along the basement membrane zone, within the lamina lucida, and localized to the proximal part of anchoring filaments of the epidermal fragment of salt-split skin)...
January 2012: Clinics in Dermatology
https://www.readbyqxmd.com/read/21955378/autoimmune-bullous-skin-diseases-part-1-clinical-manifestations
#19
REVIEW
Andrea Kneisel, Michael Hertl
Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems...
October 2011: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/21833018/black-patients-of-african-descent-and-hla-drb1-15-03-frequency-overrepresented-in-epidermolysis-bullosa-acquisita
#20
Coralie Zumelzu, Christelle Le Roux-Villet, Pascale Loiseau, Marc Busson, Michel Heller, Françoise Aucouturier, Valérie Pendaries, Nicole Lièvre, Francis Pascal, Marie-Dominique Brette, Serge Doan, Dominique Charron, Fréderic Caux, Liliane Laroche, Antoine Petit, Catherine Prost-Squarcioni
Epidermolysis bullosa acquisita (EBA) is a rare autoimmune bullous disease (AIBD). However, higher EBA incidence and predisposing genetic factor(s) involving an HLA haplotype have been suspected in some populations. This retrospective study assessed the overrepresentation of black patients with EBA, its link with HLA-DRB1*15:03, and their clinical and immunological characteristics. Between 2005 and 2009, 7/13 (54%) EBA and 6/183 (3%) other-AIBD patients seen consecutively in our department were black (P=10(-6)); moreover 7/13 (54%) black patients and 6/183 (3%) white patients had EBA (P=10(-6))...
December 2011: Journal of Investigative Dermatology
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