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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
Mirjana V Milinković, Slavenka Janković, Ljiljana Medenica, Miloš Nikolić, Vesna Reljić, Svetlana Popadić, Janko Janković
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...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Mirjana Milinković, Slavenka Janković, Ljiljana Medenica, Miloš Nikolić, Vesna Reljić, Svetlana Popadić, Janko Janković
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...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Kamran Balighi, Maryam Daneshpazhooh, Nessa Aghazadeh, Vahide Saeidi, Farzam Shahpouri, Pardis Hejazi, Cheyda Chams-Davatchi
BACKGROUND AND OBJECTIVES: Pemphigus vulgaris (PV) is typically treated with systemic corticosteroids and immunosuppressive agents. Avascular necrosis (AVN) of the femoral head is a well-recognized major complication of corticosteroid therapy. The characteristics of this serious complication in PV remain unknown. PATIENTS AND METHODS: Uncontrolled, retrospective study of all PV-related AVN cases diagnosed at an Iranian autoimmune bullous disease clinic between 1985 and 2013...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Seiko Ohno
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by degeneration of the right ventricle and ventricular tachycardia originating from the right ventricle. Additionally, the disease is an inherited cardiomyopathy that mainly follows the autosomal dominant pattern. More than 10 genes have been reported as causative genes for ARVC, and more than half of ARVC patients carry mutations in desmosome related genes. The desmosome is one of the structures involved in cell adhesion and its disruption leads to various diseases, including a skin disease called pemphigus...
October 2016: Journal of Arrhythmia
Aneliza de Fatima Moraes da Silva, Daniel Galera Bernabé, Glauco Issamu Miyahara, Eder Ricardo Biasoli, Renata Callestini, Kellen Cristine Tjioe
Oral biopsy of vesiculobullous diseases such as pemphigus vulgaris often raises questions due to some particularities involving this procedure. The adequate selection of the area to be biopsied defines if the final diagnosis will be reached, being the Achiles heel of the proper management of the patient. Here, the authors report a case of a woman who sought for treatment of generalized oral blisters and ulcers that caused severe pain. She had undergone a previous biopsy by other professionals that was inconclusive...
October 14, 2016: Journal of Craniofacial Surgery
Franziska Vielmuth, Vera Rötzer, Eva Hartlieb, Christoph Hirneiß, Jens Waschke, Volker Spindler
Purpose: The autoimmune blistering skin disease pemphigus vulgaris (PV) is caused by autoantibodies against desmosomal adhesion molecules. Patients may suffer conjunctival involvement, yet the underlying mechanisms are largely unclear. We characterized human and murine conjunctiva with respect to the PV autoantigens, and evaluated the effects and mechanisms of PV autoantibodies applied to human conjunctiva ex vivo. Methods: We obtained human conjunctiva specimens from surgical explants and established a short-term culture model to study the alterations induced by antibody fractions of PV patients (PV-IgG)...
August 1, 2016: Investigative Ophthalmology & Visual Science
Benjamin Farahnik, Collin M Blattner, Michael B Mortazie, Benjamin M Perry, William Lear, Dirk M Elston
Hailey-Hailey disease or familial benign chronic pemphigus is a rare blistering dermatosis that is characterized by recurrent erythematous plaques with a predilection for the skin folds. For extensive Hailey-Hailey disease that is recalcitrant to conventional therapy, laser ablation, photodynamic therapy, electron beam radiotherapy, botulinum toxin type A, dermabrasion, glycopyrrolate, and afamelanotide have been reported as useful treatments, but comparative trials are lacking. This review discusses the various treatment modalities for Hailey-Hailey disease and a summary of the evidence for the most recommended treatments...
October 13, 2016: Journal of the American Academy of Dermatology
V L Taliercio, B Ferrari, M E Abad, M Larralde
No abstract text is available yet for this article.
October 12, 2016: Actas Dermo-sifiliográficas
Minhee Kim, Luca Borradori, Dédée F Murrell
Elderly patients are more susceptible to the development of autoimmune blistering disorders such as bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and paraneoplastic pemphigus. This article focuses on the clinical aspects of the aforementioned autoimmune blistering diseases and highlights the important factors involved in treating elderly patients. It is essential for clinicians to offer individualized treatment plans for these patients to optimize outcomes, as elderly patients often have multiple co-morbidities, polypharmacy, and suboptimal socioeconomic status that can adversely influence adequate compliance...
October 13, 2016: Drugs & Aging
Marta Wieczorek, Annette Czernik
Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. PNP presents most frequently between 45 and 70 years old, but it also occurs in children and adolescents...
2016: Clinical, Cosmetic and Investigational Dermatology
Sangeetha Jeevan Kumar, S P Nehru Anand, Nandhini Gunasekaran, Rajkumar Krishnan
Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The initial clinical manifestation is frequently the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiology of this disease still remains obscure although the presence of autoantibodies is consistent with an autoimmune disease. These antibodies are targeted against the adhesion proteins of keratinocytes, leading to acantholysis (disruption of spinous layer, leading to intraepidermal clefting) and blister formation...
September 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
Michael Stormly Hansen, Oliver Niels Klefter, Hanne Olsen Julian, Anne Marie Lynge Pedersen, Steffen Heegaard
Pemphigoid and pemphigus diseases as well as Stevens-Johnson syndrome present as vesiculobullous disorders of the skin and may additionally involve both the oral cavity and the ocular surface. Ocular involvement ranges from mild irritation and dry eye disease to chronic conjunctivitis, symblepharon, eyelid malposition, ocular surface scarring and severe visual loss. In addition to diagnostic assessments, ophthalmologists must treat the dry eye and Meibomian gland dysfunction components of these diseases using a stepladder approach, including eyelid hygiene and lubricants...
October 7, 2016: Oral Diseases
M Kabuto, N Fujimoto, T Takahashi, T Tanaka
BACKGROUND: hile the frequency of IL-10-producing B cells (B10 cells) is reported to have an inverse correlation with disease activity in some human autoimmune diseases, the association between B10 cells and autoimmune blistering diseases (AIBD) has not been well-evaluated. Although several phenotypes of human regulatory B cells have been proposed, the most appropriate one was not established. OBJECTIVE: This study aimed to evaluate B10 cells in AIBD including their phenotypes...
October 7, 2016: British Journal of Dermatology
Sharon Baum, Assaf Debby, Sarit Gilboa, Henri Trau, Aviv Barzilai
BACKGROUND: Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease. Most patients require long-term therapy with systemic steroids, and a steroid-sparing agent is usually also utilized. Dapsone is a chemotherapeutic agent with anti-inflammatory properties that is used as a steroid-sparing agent in PV. OBJECTIVE: The aim of the present study was to evaluate the efficacy of dapsone as an adjuvant therapy in patients with PV. METHODS: A retrospective analysis of patients' files was performed...
October 4, 2016: Dermatology: International Journal for Clinical and Investigative Dermatology
Harleen Arora, Fleta N Bray, Jessica Cervantes, Leyre A Falto Aizpurua
Benign familial chronic pemphigus or Hailey-Hailey disease is caused by an autosomal dominant mutation in the ATP2C1 gene leading to suprabasilar acantholysis. The disease most commonly affects intertriginous areas symmetrically. The chronic nature of the disease and multiple recurrences make the disease bothersome for patients and a treatment challenge for physicians. Treatments include topical and/or systemic agents and surgery including laser. This review summarizes the available treatment options.
2016: Clinical, Cosmetic and Investigational Dermatology
Chander Grover, Bineeta Kashyap, Deepashree Daulatabad, Amit Dhawan, Iqbal R Kaur
BACKGROUND: Anti-cyclic citrullinated peptides (CCPs) are autoantibodies directed against citrullinated peptides. Rheumatoid factor (RF), an antibody against the Fc portion of IgG, is known to form immune complexes and contribute to the etiopathogenesis of various skin disorders. C-reactive protein (CRP), an acute-phase protein, increases following secretion of interleukin-6 from macrophages and T cells. Anti-CCP, RF, and CRP are well-established immune-markers, their diagnostic potential in immune-mediated skin disorders remains less widely studied...
September 2016: Indian Journal of Dermatology
C Günther
Autoimmune bullous diseases are characterized by intraepidermal or subepidermal autoantibody deposition that leads to blisters and secondary erosion. Mucous membranes are frequently affected in pemphigus vulgaris and always involved in cicatricial and mucosal pemphigoid. Mucosal lesions are detected less frequently in patients with bullous pemphigoid or epidermolysis bullosa acquisita. The diagnosis of autoimmune bullous disorders is based on determination of the subtype of autoantibodies bound in the skin and the clinical picture...
October 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
C Baliu-Piqué, P Iranzo
No abstract text is available yet for this article.
September 9, 2016: Actas Dermo-sifiliográficas
Sandrina Carvalho, Madalena Sanches, Rosário Alves, Manuela Selores
Pyodermatitis vegetans (PV) is a rare inflammatory dermatosis of unknown etiology and difficult diagnosis characterized by vesiculopustular, exudative, and vegetating plaques usually localized in the axillary, genital, and oral region. It bears similarities to pemphigus vegetans but clinical history, histology, and immunofluorescence help confirm the diagnosis. Pyodermatitis vegetans is often associated with an underlying gastrointestinal disorder, especially ulcerative colitis. No standardized treatment plan is available for PV...
2016: Dermatology Online Journal
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