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Pemphigus, Pemphigoid

Alessio Buonavoglia, Patrizia Leone, Rosanna Dammacco, Giuseppe Di Lernia, Massimo Petruzzi, Domenico Bonamonte, Angelo Vacca, Vito Racanelli, Franco Dammacco
Pemphigus diseases (PDs) and mucous membrane pemphigoid (MMP) are a group of immune-mediated mucocutaneous disorders clinically characterized by the formation of blisters, erosions and ulcers. The skin and mucous membranes are predominantly affected, with the oropharyngeal mucosa as the initially involved site. Ocular involvement is also a frequent feature of these diseases. Because of the considerable overlap in their clinical presentations, the diagnosis of PDs vs. MMP can be challenging. A recognition of their specific immunological and histopathologic features is crucial in the differential diagnosis...
February 7, 2019: Autoimmunity Reviews
A Welfringer-Morin, L Bekel, N Bellon, A Gantzer, O Boccara, S Hadj-Rabia, S Leclerc-Mercier, A Frassati-Biaggi, S Fraitag, C Bodemer
BACKGROUND: Autoimmune bullous dermatosis (AIBDs) in children are uncommon and their long-term evolution remains unknown. OBJECTIVE: The aim of this retrospective study was to characterize the long-term prognosis of AIBDs that started during childhood. METHODS: We conducted a monocentric retrospective study, in the French dermatology center, by including all children affected by AIBDs. The long-term outcome was obtained through a phone call questionnaire...
January 31, 2019: Journal of the European Academy of Dermatology and Venereology: JEADV
Yuichi Kurihara, Jun Yamagami, Takeru Funakoshi, Maki Ishii, Julia Miyamoto, Yumi Fujio, Risa Kakuta, Akiko Tanikawa, Yumi Aoyama, Keiji Iwatsuki, Norito Ishii, Takashi Hashimoto, Wataru Nishie, Hiroshi Shimizu, Keisuke Kouyama, Masayuki Amagai
This was a multicenter study of rituximab, a chimeric monoclonal immunoglobulin G antibody directed against CD20, for the treatment of refractory autoimmune bullous diseases (pemphigus and pemphigoid). Ten patients (three with pemphigus vulgaris, six with pemphigus foliaceus and one with bullous pemphigoid) were treated with a single cycle of rituximab (four weekly infusions at a dose of 375 mg/m2 of body surface area). The primary end-points were the number of serious adverse events and rate of complete remission at 40 weeks...
December 26, 2018: Journal of Dermatology
Aikaterini Patsatsi, Aikaterini Kyriakou, Victoria P Werth
Bullous pemphigoid (BP) is the most common autoimmune blistering disease affecting the elderly but is quite rare in childhood. The majority of pediatric cases have been reported during early childhood. Adolescence is divided into three phases: early (10-13 years), middle (14-17), and late (18-21). This review aimed to identify BP cases in adolescence and demonstrate their clinical features and course. Our literature search was performed in Medline with the terms "bullous pemphigoid in childhood and adolescence," "childhood bullous pemphigoid," "juvenile bullous pemphigoid," and "autoimmune blistering and autoimmune bullous diseases in childhood...
December 19, 2018: Pediatric Dermatology
Mauro Alaibac
Autoimmune blistering skin diseases are a group of disorders subdivided according to the location of blister formation: intraepidermal blistering in the pemphigus group and subepidermal in the pemphigoid group. These conditions are clinically heterogeneous and are treated with systemic corticosteroids and/or other forms of immunosuppression on the basis of clinical subtype and disease severity. These approaches may not be effective for the induction and maintenance of clinical response or need to be stopped because of intolerable side effects...
December 18, 2018: Expert Opinion on Biological Therapy
Eden Lake
The incidence of zoster is increased in many autoimmune diseases. This study found a significantly increased zoster incidence in dermatomyositis and cutaneous lupus erythematosus compared with a control group.Physicians should be aware of this risk and consider administering the vaccine against zoster to these patients if not contraindicated.
December 3, 2018: Journal of the American Academy of Dermatology
Jinmin Lee, Victoria P Werth, Russell P Hall, Rüdiger Eming, Janet A Fairley, David C Fajgenbaum, Karen E Harman, Marcel F Jonkman, Neil J Korman, Ralf J Ludwig, Dedee F Murrell, Philippe Musette, Haley B Naik, Christian D Sadik, Jun Yamagami, Marc L Yale, Aimee S Payne
The 5th Scientific Conference of the International Pemphigus and Pemphigoid Foundation (IPPF), "Pemphigus and Pemphigoid: A New Era of Clinical and Translational Science" was held in Orlando, Florida, on May 15-16, 2018. Scientific sessions covered recent, ongoing, and future clinical trials in pemphigus and bullous pemphigoid, disease activity and quality of life instruments, and the IPPF Natural History Study. Furthermore, the meeting provided an opportunity to hear firsthand from patients, investigators, and industry about their experience enrolling for clinical trials...
2018: Frontiers in Medicine
Mareike Witte, Detlef Zillikens, Enno Schmidt
Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Circulating antibodies can be detected via indirect immunofluorescence (IIF) microscopy of different tissue substrates including human skin, monkey esophagus, and more recently, recombinant forms of the different target antigens...
2018: Frontiers in Medicine
Khalaf Kridin, Reuven Bergman
The specificity and the predictive values of indirect immunofluorescence (IIF) in real-life settings is yet to be firmly established. The natural history of patients with false-positive results has not been sufficiently elucidated. The primary aim of the current study is to evaluate the diagnostic value of IIF analysis on monkey esophagus in pemphigus, utilizing a large cohort arising from the real-life experience of a tertiary referral center. The secondary endpoint was to determine the clinical outcomes of patients with false-positive results...
2018: Frontiers in Medicine
Claudio Feliciani, Giovanni Genovese, Roberto D'astolto, Paolo Pontini, Angelo V Marzano
Pemphigoid gestationis (PG), also known as herpes gestationis, is the prototypic pregnancy-associated autoimmune bullous disease (AIBD), but also the other AIBDs, notably pemphigus vulgaris, may begin or exacerbate during pregnancy. Although the increase in concentration of T and B regulatory cells makes pregnancy a state of increased immunologic tolerance toward the semiallogeneic fetal antigens, a prevalent T helper (Th) 2 profile, that is reported to be associated with pregnancy, may cause exacerbation of pemphigus and AIBDs in general during this period...
October 29, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
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...
October 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: Antilaminin 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; however, no standardized detection system for antilaminin 332 serum antibodies is widely available. OBJECTIVES: Development of a sensitive and specific assay for the detection of antilaminin 332 antibodies. METHODS: An indirect immunofluorescence (IF) assay using recombinant laminin 332 was developed and probed with a large number of antilaminin 332 MMP patient sera (n = 93), as well as sera from patients with antilaminin 332-negative MMP (n = 153), bullous pemphigoid (n = 20), pemphigus vulgaris (n = 20) and noninflammatory 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...
January 2019: International Journal of Dermatology
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