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Pemphigus, Pemphigoid

R Maglie, M Hertl
No abstract text is available yet for this article.
October 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Kyle T Amber, Roberto Maglie, Farzan Solimani, Rüdiger Eming, Michael Hertl
Autoimmune bullous skin disorders are rare but meaningful chronic inflammatory diseases, many of which had a poor or devastating prognosis prior to the advent of immunosuppressive drugs such as systemic corticosteroids, which down-regulate the immune pathogenesis in these disorders. Glucocorticoids and adjuvant immunosuppressive drugs have been of major benefit for the fast control of most of these disorders, but their long-term use is limited by major side effects such as blood cytopenia, osteoporosis, diabetes mellitus, hypertension, and gastrointestinal ulcers...
September 20, 2018: Drugs
Jun Yamagami
Pemphigus and pemphigoid are characterized as autoimmune blistering diseases in which immunoglobulin G autoantibodies cause blisters and erosions of the skin or mucosa or both. Recently, understanding of the pathophysiology of pemphigus and pemphigoid has been furthered by genetic analyses, characterization of autoantibodies and autoreactive B cells, and elucidation of cell-cell adhesion between keratinocytes. For the management of pemphigus and pemphigoid, the administration of systemic corticosteroids still represents the standard treatment strategy; however, evidence of the efficacy of therapies not involving corticosteroids, such as those employing anti-CD20 antibodies, is increasing...
2018: F1000Research
S Goletz, C Probst, L Komorowski, W Schlumberger, K Fechner, N van Beek, M M Holtsche, A Recke, K B Yancey, T Hashimoto, F Antonicelli, G Di Zenzo, D Zillikens, W Stöcker, E Schmidt
BACKGROUND: Anti-laminin 332 mucous membrane pemphigoid (MMP) is an autoimmune subepidermal blistering disease with predominant mucosal involvement and autoantibodies against laminin 332. Malignancies have been associated with this disease but however, no standardized detection system for anti-laminin 332 serum antibodies is widely available. OBJECTIVE: Development of a sensitive and specific assay for the detection of anti-laminin 332 antibodies. METHODS: An indirect immunofluorescence (IF) assay using recombinant laminin 332 was developed and probed with a large number of anti-laminin 332 MMP patients sera (n=93) as well as sera from patients with anti-laminin 332-negative MMP (n=153), bullous pemphigoid (n=20), pemphigus vulgaris (n=20), non-inflammatory dermatoses (n=22), and healthy blood donors (n=100)...
September 14, 2018: British Journal of Dermatology
Istvan A Hargitai
Painful oral vesiculoerosive diseases (OVD) include lichen planus, pemphigus vulgaris, mucous membrane pemphigoid, erythema multiforme, and recurrent aphthous stomatitis. OVD lesions have an immunopathic cause. Treatment is aimed at reducing the immunologic and the following inflammatory response. The mainstay of OVD management is topical or systemic corticosteroids to include topical triamcinolone, fluocinonide, and clobetasol, whereas systemic medications used in practice can include dexamethasone, prednisone, and prednisolone...
October 2018: Dental Clinics of North America
Nina van Beek, Detlef Zillikens, Enno Schmidt
Blasenbildende Autoimmundermatosen (BAIDs) sind eine heterogene Gruppe seltener Erkrankungen, die klinisch durch Erosionen und/oder Blasen an Haut und Schleimhäuten charakterisiert sind. BAIDs können in zwei Gruppen eingeteilt werden: Pemphigus-Erkrankungen, die durch intraepidermale Blasenbildung und Autoantikörper gegen desmosomale Proteine wie Desmoglein (Dsg) 1, Dsg3 und Mitglieder der Plakin-Familie charakterisiert sind, sowie subepidermale BAIDs, die Pemphigoid-Erkrankungen und die Dermatitis herpetiformis umfassen...
September 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Nina van Beek, Detlef Zillikens, Enno Schmidt
Autoimmune bullous disorders (AIBDs) are a heterogeneous group of rare diseases clinically characterized by erosions and/or blisters on the skin and mucous membranes. AIBDs can be categorized into two groups: pemphigus diseases, characterized by intraepidermal blistering and autoantibodies against desmosomal proteins such as desmoglein (Dsg) 1, Dsg3, members of the plakin family, and subepidermal AIBDs, comprised of pemphigoid diseases and dermatitis herpetiformis. Autoantibodies in dermatitis herpetiformis target transglutaminases 2 and 3, while in pemphigoid disease, autoantibodies are directed against structural proteins of the dermal-epidermal junction...
September 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Franziska Hübner, Michael Kasperkiewicz, Diana Knuth-Rehr, Iakov Shimanovich, Joachim Hübner, Sven Süfke, Philip Muck, Detlef Zillikens, Enno Schmidt
HINTERGRUND: Mittels Immunadsorption (IA) können Immunglobuline und Immunkomplexe aus dem Plasma entfernt werden. Während dieses therapeutische Verfahren beim Pemphigus vulgaris bereits etabliert ist, wird es bei anderen blasenbildenden Autoimmundermatosen bislang nur sporadisch eingesetzt. Das bullöse Pemphigoid (BP) spricht zwar meist gut auf eine Therapie mit topischen und systemischen Kortikosteroiden an, jedoch könnte bei Patienten mit ausgedehnten Läsionen oder bei einem Rezidiv die rasche Reduktion der pathogenen Autoantikörper eine effektive adjuvante Therapie darstellen...
September 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Franziska Hübner, Michael Kasperkiewicz, Diana Knuth-Rehr, Iakov Shimanovich, Joachim Hübner, Sven Süfke, Philip Muck, Detlef Zillikens, Enno Schmidt
BACKGROUND: While depletion of circulating autoantibodies using immunoadsorption (IA) is an established therapeutic approach in patients with pemphigus vulgaris, IA has only sporadically been used in other autoimmune bullous disorders. Although bullous pemphigoid (BP) usually responds well to topical and systemic corticosteroids, rapid depletion of serum autoantibodies may be an effective adjuvant treatment option in patients with severe and/or refractory disease. PATIENTS AND METHODS: Case series of 20 patients (13 women, 7 men; mean age 78...
September 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Pamela N Madu, Victoria L Williams, Megan H Noe, Bernard G Omech, Carrie L Kovarik, Karolyn A Wanat
BACKGROUND: There is a paucity of data describing autoimmune skin diseases in sub-Saharan Africa and in HIV positive cohorts. We describe the incidence of autoimmune skin diseases in public dermatology clinics in Botswana. METHODS: New patient records from public dermatology clinics were reviewed retrospectively for the period of September 2008 to December 2015. New diagnoses of cutaneous lupus erythematosus, systemic lupus erythematosus (SLE) with cutaneous involvement, dermatomyositis, systemic sclerosis, lichen sclerosus, bullous pemphigoid, pemphigus foliaceus, pemphigus vulgaris, and pemphigus vegetans were identified...
August 31, 2018: International Journal of Dermatology
S-M Dridi, F Bellakhdar, N Ortonne, K Bayet, S Ingen-Housz-Oro, F Gaultier
BACKGROUND: Gingival expression of autoimmune bullous diseases (AIBD) may be inaugural, exclusive or dominant (mucous membrane pemphigoid, pemphigus vulgaris). Histology and direct immunofluorescence are essential to diagnosis. The location of the biopsy and the surgical technique determine the histological quality of the tissue sample. However, gingival tissue is often considered fragile and easily impaired during biopsy. We suggest an original biopsy protocol for the gingival papillae that is simple to perform, non-iatrogenic, and readily accessible to all practitioners who usually treat AIBD patients presenting isolated gingival expression (dermatologists, stomatologists, odontology specialists, ENT specialists)...
October 2018: Annales de Dermatologie et de Vénéréologie
Michael McFarlane, Ayesha Azam, David Snead, Ben Disney
Pemphigus vulgaris (PV) is a rare autoimmune bullous disease which affects the skin and mucous membranes. Oesophageal involvement is rare and has previously been limited to case reports and case series. A recent large case series of 477 PV patients showed that 26/477 (5.4%) had symptomatic oesophageal involvement. We present the case of a 54-year-old Somalian lady with a 10-year history of cutaneous PV, currently in remission, who developed dysphagia and odynophagia and was subsequently found to have oesophageal PV involvement with multiple flaccid bullae which were positive for anti-DSG3 antibodies on in-direct immunofluorescence...
August 23, 2018: Clinical Journal of Gastroenterology
Andreas Recke, Sarah Konitzer, Susanne Lemcke, Miriam Freitag, Nele Maxi Sommer, Mohammad Abdelhady, Mahsa M Amoli, Sandrine Benoit, Farha El-Chennawy, Mohammad Eldarouti, Rüdiger Eming, Regine Gläser, Claudia Günther, Eva Hadaschik, Bernhard Homey, Wolfgang Lieb, Wiebke K Peitsch, Claudia Pföhler, Reza M Robati, Marjan Saeedi, Miklós Sárdy, Michael Sticherling, Soner Uzun, Margitta Worm, Detlef Zillikens, Saleh Ibrahim, Gestur Vidarsson, Enno Schmidt
IgG3 is the IgG subclass with the strongest effector functions among all four IgG subclasses and the highest degree of allelic variability among all constant immunoglobulin genes. Due to its genetic position, IgG3 is often the first isotype an antibody switches to before IgG1 or IgG4. Compared with the other IgG subclasses, it has a reduced half-life which is probably connected to a decreased affinity to the neonatal Fc receptor (FcRn). However, a few allelic variants harbor an amino acid replacement of His435 to Arg that reverts the half-life of the resulting IgG3 to the same level as the other IgG subclasses...
2018: Frontiers in Immunology
Naiyu Lin, Qingxiu Liu, Menglei Wang, Qian Wang, Kang Zeng
Background: Pemphigus is a common life-threatening, autoimmune bullous disease effecting both cutaneous and mucous membranes. Previous diagnosis of pemphigus is based on clinical presentations, histopathology, immunofluorescence and enzyme-linked immunosorbent assay. Furthermore, no laboratory parameters could be used to indicate disease severity. MicroRNAs are endogenous small RNAs, which could be used as diagnostic biomarkers for some autoimmune diseases. Previously, miR-338-3p has been proven significantly up-regulated in pemphigus patients...
2018: PeerJ
Matteo Saccucci, Gabriele Di Carlo, Maurizio Bossù, Francesca Giovarruscio, Alessandro Salucci, Antonella Polimeni
Oral signs are frequently the first manifestation of autoimmune diseases. For this reason, dentists play an important role in the detection of emerging autoimmune pathologies. Indeed, an early diagnosis can play a decisive role in improving the quality of treatment strategies as well as quality of life. This can be obtained thanks to specific knowledge of oral manifestations of autoimmune diseases. This review is aimed at describing oral presentations, diagnosis, and treatment strategies for systemic lupus erythematosus, Sjögren syndrome, pemphigus vulgaris, mucous membrane pemphigoid, and Behcet disease...
2018: Journal of Immunology Research
Enke Baldini, Teresa Odorisio, Chiara Tuccilli, Severino Persechino, Salvatore Sorrenti, Antonio Catania, Daniele Pironi, Giovanni Carbotta, Laura Giacomelli, Stefano Arcieri, Massimo Vergine, Massimo Monti, Salvatore Ulisse
The skin is the largest organ of the body, at the boundary with the outside environment. Primarily, it provides a physical and chemical barrier against external insults, but it can act also as immune organ because it contains a whole host of immune-competent cells of both the innate and the adaptive immune systems, which cooperate in eliminating invading pathogens following tissue injury. On the other hand, improper skin immune responses lead to autoimmune skin diseases (AISD), such as pemphigus, bullous pemphigoid, vitiligo, and alopecia...
June 15, 2018: Reviews in Endocrine & Metabolic Disorders
Wei-Shen Chen, Michael T Tetzlaff, Hafeez Diwan, Richard Jahan-Tigh, Adi Diab, Kelly Nelson, Isabella C Glitza, Genevieve J Kaunitz, Daniel Johnson, Carlos Torres-Cabala, Omar Pacha, Janis M Taube, Kudakwashe Maloney, Victor G Prieto, Jonathan L Curry
Checkpoint inhibitors (CPIs) restore the function of effector immunocytes to target and destroy cancer cells. Immune-related adverse events (irAEs) are a consequence of immune reactivation, with unpredictable inflammatory response, loss of self-tolerance, and development of autoimmunity. Adverse events from CPIs that present as dermatologic toxicities have diverse clinical and histopathologic features. CPI-associated dermatologic toxicities may exhibit histopathologic features of lichenoid dermatitis, bullous pemphigoid, and granulomatous/sarcoid-like reactions...
October 2018: Journal of Cutaneous Pathology
Swastika Suvirya, Sucheta Pathania, Kiran Preet Malhotra, Parul Verma
Cicatricial pemphigoid (CP) is a chronic, autoimmune, subepidermal blistering disease with predominant mucosal involvement. In this article, we report a young patient with mucosal and extensive cutaneous involvement in the form of large erosions mimicking those of pemphigus vulgaris thus leading to diagnostic dilemma. We were unable to find any other previous reports with such extensive cutaneous erosions mimicking those of pemphigus vulgaris. Laminin 5 was the antigen found on knockout substrate testing. Antiepiligrin CP is a distinct subtype of CP with antibodies against laminin 5...
June 21, 2018: BMJ Case Reports
Oscar Thabouillot, Julien Le Coz, Nicolas-Charles Roche
A seven-month-old girl was referred to the emergency department (ED) after a general practitioner suspected Steven-Johnson syndrome. Actually, the diagnosis of bullous pemphigoid (BP) was made based on biopsies; BP is a rare, autoimmune skin disease involving the presence of blisters known as bullae. The child was efficiently treated with topical steroids. This case shows the importance of the ED physician's prior knowledge of BP so that a differential diagnosis with other autoimmune diseases (dermatosis, pemphigus) can be made...
August 2018: Prehospital and Disaster Medicine
Atena Shirzad, Ali Bijani, Mahsa Mehryari, Mina Motallebnejad, Saman Mohsenitavakoli
Background: Chronic oral mucosal disease q uestionnaire (COMDQ) is used to evaluate the quality of life in patients with chronic conditions of the oral mucosa. The aim of the present study was to evaluate the validity and reliability of the Persian version of this questionnaire. Methods: A total of 135 subjects were selected in two groups; group 1 consisted of 95 patients with chronic oral mucosal conditions, including recurrent aphthous stomatitis, oral lichen planus and pemphigus and mucous membrane pemphigoid and group 2 consisted of 40 patients with other oral diseases...
2018: Caspian Journal of Internal Medicine
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