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Pemphigus

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https://www.readbyqxmd.com/read/27914107/non-paraneoplastic-autoimmune-subepidermal-bullous-disease-associated-with-fatal-bronchiolitis-obliterans
#1
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/27902996/pemphigus-pathogenesis-to-treatment
#2
Christopher DiMarco
Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. These lesions correlate histologically with splits forming in the epidermis, leaving a blister roof composed of a few cell layers...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27881029/pemphigus-herpetiformis-type-drug-reaction-caused-by-erdosteine-containing-mucolytic-in-a-child
#3
Gulsen Akoglu, Sibel Orhun Yavuz
Drug-related pemphigus is very rare in children. Erdosteine is a thiol compound having mucoactive, antioxidant, anti-inflammatory, and antitussive effects and is reported to be safe for treatment of acute respiratory tract diseases in children. Herein, we report a 9-year-old boy presented with pemphigus herpetiformis associated with anti-desmoglein 1 antibodies due to erdosteine consumption.
November 23, 2016: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/27879012/crusted-impetigo-like-lesion-on-the-face-a-case-of-igg-iga-pemphigus
#4
R Moriuchi, T Ito, K Kikuchi, N Nakashita, R Muramatsu, K Natsuga, H Shimizu, S Shimizu
In recent years, cases of IgG/IgA pemphigus, a rare subtype of pemphigus characterized by circulating IgG and IgA autoantibodies against the keratinocyte cell surface have been reported.(1-6) The cutaneous manifestations of IgG/IgA pemphigus were reported to resemble those of IgA pemphigus in form, including the presence of pustules and annular lesions.(1) However, no consensus on what eruptions are typical of IgG/IgA pemphigus has been established, due to the rarity of this disease. In this report, we describe a case of IgG/IgA pemphigus who developed severe crusted lesions on the seborrheic area that closely resembled facial crusted impetigo...
November 23, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27878477/paraneoplastic-pemphigus-and-autoimmune-blistering-diseases-associated-with-neoplasm-characteristics-diagnosis-associated-neoplasms-proposed-pathogenesis-treatment
#5
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
https://www.readbyqxmd.com/read/27872890/koebner-phenomenon-in-pemphigus-vulgaris-patients
#6
Kamran Balighi, Maryam Daneshpazhooh, Arghavan Azizpour, Vahide Lajevardi, Fariba Mohammadi, Cheyda Chams-Davatchi
No abstract text is available yet for this article.
September 2016: JAAD Case Reports
https://www.readbyqxmd.com/read/27871350/early-description-of-diet-induced-blistering-skin-diseases-in-medieval-persia-avicenna-s-point-of-view
#7
Fatemeh Atarzadeh, Babak Daneshfard, Ladan Dastgheib, Amir-Mohammad Jaladat, Gholamreza Amin
Pemphigus is an autoimmune blistering skin disease that is strongly associated with different environmental factors. Among these, nutritional factors are considered to trigger pemphigus; however, their role may be underestimated. Investigated more recently in conventional medicine, this causative bond between dietary factors and blistering skin diseases was mentioned by Persian scholars such as Avicenna a thousand years ago. Avicenna, a well-known Persian physician and philosopher, who could be considered a pioneer in dermatology, discussed skin diseases in a chapter in The Canon of Medicine...
2016: Skinmed
https://www.readbyqxmd.com/read/27869434/-childhood-pemphigus-vulgaris-a-case-report
#8
Ana G Bellelli, Natalia M Mantero, María L Rueda, Daniel Navacchia, Gabriel Cao, Leonardo De Lillo, Susana A Grees
Pemphigus vulgaris is a chronic disease that is characterized by flaccid blisters and erosions, caused by immunoglobulin G autoantibodies against epidermal components. It is highly unusual in the pediatric population; it represents the 1.4 - 2.9% of the total cases. We present a case of pemphigus vulgaris in a 12 year old girl, which has implied a challenge both in its diagnosis and in its treatment.
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27867744/iga-pemphigus-showing-iga-antibodies-to-desmoglein-1-and-3
#9
Salama Hegazy, Sana Bouchouicha, Aida Khaled, Lilia Laadher, Maryem Kallel Sellami, Faten Zeglaoui
BACKGROUND: IgA pemphigus is a rare autoimmune vesiculo-pustular skin disease. Only approximately 70 cases have been reported to date. We report a case of IgA pemphigus with IgA antibodies to desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). CASE REPORT: We report the case of an 60-year-old man with intraepidermal neutrophilic IgA pemphigus with IgA antibodies to Dsg1 and Dsg3. Histologic examination revealed subcorneal neutrophilic pustules with few acantholytic cells...
October 2016: Dermatology Practical & Conceptual
https://www.readbyqxmd.com/read/27862562/management-of-pemphigus-disease-in-pregnancy
#10
REVIEW
Soheil Tavakolpour, Hajar Sadat Mirsafaei, Saeid Delshad
Pemphigus can cause complications during pregnancy and may cause serious harm to a fetus. For this study, a comprehensive review of common treatments of pemphigus and their adverse effects associated with pregnancy and male fertility was conducted. We concluded that a period of remission with minimal or no therapy before conception could significantly reduce the risk of the disease flaring up, at least in the first trimester. The period of remission causes a delay in the flare-up of the disease, which means lower cumulative doses and the prevention of possible congenital abnormalities caused by corticosteroid or immunosuppressant treatments...
November 8, 2016: American Journal of Reproductive Immunology: AJRI
https://www.readbyqxmd.com/read/27855302/the-possible-role-of-interleukin-35-and-its-therapeutic-potential-in-pemphigus
#11
REVIEW
Soheil Tavakolpour, Forough Kheiry, Hajar Sadat Mirsafaei, Meisam Akhlaghdoust
Pemphigus is an autoimmune disease that causes blistering and is life-threatening if left untreated. Nowadays, finding a promising treatment for pemphigus remains a serious challenge. Various treatments are currently recommended to treat this disease, but they rarely lead to complete and durable remission. Regulatory cells appear to have a critical role in numerous autoimmune diseases, so it is possible that promotion of these cells may induce remission. This study presents a new approach to treating pemphigus that has not been discussed to date...
November 14, 2016: International Immunopharmacology
https://www.readbyqxmd.com/read/27829102/correlation-of-serum-levels-of-ige-autoantibodies-against-bp180-with-bullous-pemphigoid-disease-activity
#12
Nina van Beek, Nadine Lüttmann, Franziska Huebner, Andreas Recke, Ingolf Karl, Franziska Sophie Schulze, Detlef Zillikens, Enno Schmidt
Importance: Bullous pemphigoid (BP) is by far the most frequent autoimmune blistering disease. The presence of IgE autoantibodies against the transmembrane protein BP antigen 2 (BP180, type XVII collagen) has previously been reported in 22% to 100% of BP serum samples, and the pathogenic relevance of anti-BP180 IgE has been suggested in various experimental models and by the successful use of omalizumab in individual patients with BP. Objectives: To determine the rate of anti-BP180-reactive IgE in BP, to evaluate the diagnostic relevance of anti-BP180 IgE in BP, and to correlate anti-BP180 IgE with disease activity and the clinical phenotype of patients with BP...
November 9, 2016: JAMA Dermatology
https://www.readbyqxmd.com/read/27828647/generalized-pustular-psoriasis-induced-by-systemic-steroid-dose-reduction
#13
Danielle Cristine Westphal, Antonio Pedro Mendes Schettini, Petra Pereira de Souza, Jessica Castiel, Carlos Alberto Chirano, Mônica Santos
Generalized pustular psoriasis, or psoriasis of von Zumbusch, is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this work, we report the case of a female patient, 70 years old, who developed generalized pustular psoriasis symptoms while reducing the dose of oral corticosteroids, improperly introduced for the treatment of alleged acute generalized exanthematous pustulosis. The differential diagnosis of generalized pustular psoriasis should be made with other pustular dermatoses, such as subcorneal pustulosis, IgA pemphigus and especially with acute generalized exanthematous pustulosis...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27826815/is-there-a-role-for-opportunistic-infection-prophylaxis-in-pemphigus-an-expert-survey
#14
Yael A Leshem, Igor Snast, Rivka Friedland, Lihi Atzmony, Assi Levi, Emmilia Hodak, Daniel Mimouni
BACKGROUND: Opportunistic infections (OIs) are a dreaded adverse effect of immunosuppressive therapy, leading to the use of opportunistic infection prophylaxis (OIP) in many immunosuppressed conditions. However, guidelines for OIP in pemphigus are lacking. OBJECTIVE: Our objective was to evaluate the approach of leading pemphigus experts towards OIP. METHODS: We conducted an online survey of OIP trends. RESULTS: The survey was completed by 33 experts...
November 8, 2016: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/27814654/a-reciprocal-hla-disease-association-in-rheumatoid-arthritis-and-pemphigus-vulgaris
#15
Vincent Van Drongelen, Joseph Holoshitz
  Human leukocyte antigens (HLA) have been extensively studied as being antigen presenting receptors, but many aspects of their function remain elusive, especially their association with various autoimmune diseases. Here we discuss an illustrative case of the reciprocal relationship between certain HLA-DRB1 alleles and two diseases, rheumatoid arthritis (RA) and pemphigus vulgaris (PV). RA is strongly associated with HLA-DRB1 alleles that encode a five amino acid sequence motif in the 70-74 region of the DR beta chain, called the shared epitope (SE), while PV is associated with the HLA-DRB1*04:02 allele that encodes a different sequence motif in the same region...
January 1, 2017: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/27812271/peri-anal-paraneoplastic-pemphigus-heralding-the-relapse-of-follicular-lymphoma-and-its-successful-management-by-rituximab-a-short-correspondence
#16
Ankur Jain, Gaurav Prakash, Ram V Nampoothiri, Dipankar De, Amanjit Bal, Alka Khadwal, Deepesh Lad, Pankaj Malhotra, Subhash Varma
No abstract text is available yet for this article.
December 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27800178/the-prevalence-of-s-aureus-skin-and-soft-tissue-infections-in-patients-with-pemphigus
#17
Zeinab Fagheei Aghmiyuni, Ahmad Khorshidi, Rezvan Moniri, Tahereh Soori, Seyed Gholam Abbas Musavi
Pemphigus vulgaris are autoimmune blistering diseases that may result in significant morbidity and death. Immunosuppressive therapy of pemphigus vulgaris would predispose the patients to infections. The aim of this study was to assess the prevalence of S. aureus infection and PVL gene in patients with pemphigus admitted to dermatology clinic. Materials and Methods. This descriptive study was conducted on 196 pemphigus vulgaris patients (119 males, 77 females) admitted to dermatology clinic between 2014 and 2015...
2016: Autoimmune Diseases
https://www.readbyqxmd.com/read/27796136/rituximab-treatment-of-pemphigus-in-women-of-childbearing-age-experience-with-two-patients
#18
Eden P Lake, Yu-Hui Huang, Iris K Aronson
Pemphigus vulgaris is an autoimmune blistering disorder treated with systemic steroids and immunosuppressive agents. Treatment of this disorder in young women of childbearing age must take into consideration the patient's desire for pregnancy and effects of the treatment on both mother and child. We report two young women with pemphigus, initially treated with standard immunosuppressive medications, who expressed their wishes for pregnancy. The immunosuppressive agents were tapered and both patients were treated with Rituximab and IVIG, permitting discontinuation of other medications, conception and pregnancy without any oral steroids or immunosuppressive agents...
November 20, 2016: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/27792691/-hla-class-ii-in-mexican-patients-with-pemphigus-vulgaris-shared-epitope-for-autoimmunity
#19
Lucía Rangel-Gamboa, María Elisa Vega-Memije, Víctor Acuña-Alonzo, Julio Granados-Arriola
INTRODUCTION: Pemphigus is an autoimmune blistering disease of skin and mucous membranes characterized by presence of IgG antibodies against desmoglein 3, and 1. Desmoglein 3 and 1 are presented in pemphigus vulgaris and pemphigus foliaceous, respectively. Desmoglein are transmembrane proteins that form part of cellular junctions called desmosomes. Major histocompatibility complex class II molecules have been related to autoimmune disease; in pemphigus vulgaris, different human lymphocyte antigens (HLA) were associated among different ethnic groups, such as HLA-DR4, HLA-DR14, and HLA-DR1...
September 2016: Gaceta Médica de México
https://www.readbyqxmd.com/read/27767277/zeitlicher-verlauf-der-avaskul%C3%A3-ren-nekrose-des-h%C3%A3-ftkopfes-bei-patienten-mit-pemphigus-vulgaris
#20
Kamran Balighi, Maryam Daneshpazhooh, Nessa Aghazadeh, Vahide Saeidi, Farzam Shahpouri, Pardis Hejazi, Cheyda Chams-Davatchi
HINTERGRUND UND ZIELE: Pemphigus vulgaris (PV) wird in der Regel mit systemischen Corticosteroiden und Immunsuppressiva behandelt. Avaskuläre Nekrose (AVN) des Hüftkopfes ist eine gut bekannte schwerere Komplikation einer Corticosteroid-Therapie. Die Charakteristika dieser schweren Komplikation bei PV sind nach wie vor unbekannt. PATIENTEN UND METHODEN: Nicht kontrollierte, retrospektive Untersuchung aller PV-bedingten AVN-Fälle, die in einer iranischen Klinik für bullöse Autoimmunerkrankungen zwischen 1985 und 2013 diagnostiziert wurden...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
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