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K L Wang, Y P Xie, Y Xu, P F Shi, S X Qian
No abstract text is available yet for this article.
February 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Zhanli Fu, Meng Liu, Xueqi Chen, Xing Yang, Qian Li
A 36-year-old woman suffering from oral ulcerations, skin rash, and dyspnea was suspected of having paraneoplastic pemphigus. An F-FDG PET/CT was performed to detect the underlying neoplasms and revealed an F-FDG-avid mass in the lower right retroperitoneum. The mucocutaneous lesions and dyspnea were relieved after the tumor excision, and the pathology indicated Castleman disease.
March 20, 2018: Clinical Nuclear Medicine
Thanisorn Sukakul, Supenya Varothai
Pemphigus vegetans is a rare variant of deep acantholytic pemphigus which usually presents with vesiculobullous rash and vegetative plaques on the folds. We report a case of pemphigus vegetans patient who presented with rashes on tips of fingers and toes resembling paronychia and onychomadesis that misled the diagnosis for months. The final diagnosis of Hallopeau-type pemphigus vegetans was made based on histopathology and direct immunofluorescence studies. Interestingly, not only the clinical presentation was atypical, but blood tests for anti-desmoglein 1 and 3 antibodies by ELISA technique were also negative...
2018: Case Reports in Medicine
Fuqiong Jiang, Danqi Deng, Xiaolan Li, Wenfang Wang, Hong Xie, Yongzhuo Wu, Chunyan Luan, Binbin Yang
To determine clinical curative effects of ozone therapy for pemphigus vulgaris.
 Methods: Ozone hydrotherapy was used as an aid treatment for 32 patients with pemphigus vulgaris. The hydropathic compression of potassium permanganate solution for 34 patients with pemphigus vulgaris served as a control. The main treatment for both groups were glucocorticoids and immune inhibitors. The lesions of patients, bacterial infection, usage of antibiotics, patient's satisfaction, and clinical curative effect were evaluated in the 2 groups...
February 28, 2018: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Anissa Zaouak, Houda Hammami, Mariem Belhaj Salah, Achraf Debbiche, Samy Fenniche
A 58-year-old man presented to our department with multiple chronic vegetant plaques on the face and scalp that had been present for 4 months. The plaques were well-defined hypertrophic areas, measuring between 3 cm × 3 cm and 3 cm × 4 cm, that were located mainly on the face symmetrically and on the forehead (Figure 1). The patient did not report itching or pain. There were also dry, crusty, well-defined plaques on the scalp. No other lesions were present on the skin or mucous membranes.
2018: Skinmed
Jianbo Chen, Yao Zhang, Yunsheng Liang, Ming Zhao, Hai Long, Rong Xiao, Haijing Wu, Jieyue Liao, Shuaihantian Luo, Guiying Zhang, Qianjin Lu
Pemphigus vulgaris is an autoimmune blistering disease characterized by a loss of epidermal cell-cell adhesion caused by anti-desmoglein (Dsg) autoantibodies. The pathogenesis of PV remains unclear. However, abnormal frequency and function of Th2 cells are believed to contribute to PV. We investigated Nr4a2, a transcription factor, which has been found to regulate T cell differentiation, for its association with Th2 cell differentiation and functions in PV. We found significantly decreased mRNA and protein levels of Nr4a2 in CD4+ T cells from patients with PV, compared with healthy control subjects...
February 16, 2018: Oncotarget
Sivaranjini Ramassamy, Poonam Agrawal, Dharshini Sathishkumar, Lydia Mathew, John Victor Peter, Thenmozhi Mani, Renu George
BACKGROUND: Pemphigus has a protracted course and multiple factors influence its prognosis. The objective of this study was to describe the epidemiology and clinical profile of pemphigus patients and to study its influence on treatment end points. METHODS: This was a retrospective chart review done in an Indian tertiary care hospital from December 1991 to December 2013. Patients with less than 3 months' follow up and those who had paraneoplastic pemphigus were excluded...
March 12, 2018: Indian Journal of Dermatology, Venereology and Leprology
Roberta Lotti, En Shu, Tiziana Petrachi, Alessandra Marconi, Elisabetta Palazzo, Marika Quadri, Ann Lin, Lorraine A O'Reilly, Carlo Pincelli
Pemphigus is a blistering disease characterized by pemphigus autoantibodies (PVIgG) directed mostly against desmogleins (Dsgs), resulting in the loss of keratinocyte adhesion (acantholysis). Yet, the mechanisms underlying blister formation remain to be clarified. We have shown previously that anti-Fas ligand (FasL) antibody (Ab) prevents PVIgG-induced caspase-8 activation and Dsg cleavage in human keratinocytes, and that sera from pemphigus patients contain abnormally increased levels of FasL. Here, we demonstrate that recombinant FasL induces the activation of caspases prior to Dsg degradation, and anti-FasL Ab prevents acantholysis in cultured keratinocytes...
2018: Frontiers in Immunology
Yuta Ito, Shinichi Makita, Akiko Miyagi Maeshima, Shunsuke Hatta, Tomotaka Suzuki, Sayako Yuda, Suguru Fukuhara, Wataru Munakata, Tatsuya Suzuki, Dai Maruyama, Koji Izutsu
Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease associated with an underlying malignancy, and its prognosis is poor. We herein report the first patient with B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL)-associated PNP successfully treated with the Bruton's tyrosine kinase inhibitor ibrutinib and rituximab. Although his PNP lesions did not improve with ibrutinib monotherapy, the combination of ibrutinib and rituximab was effective against B-CLL/SLL-associated PNP...
March 9, 2018: Internal Medicine
Mohamed Hussein Medhat El-Komy, Noha Adly Saleh, Marwah Adly Saleh
Oral erosions of pemphigus vulgaris (PV) represent a therapeutic challenge. In this work we compared intralesional injection of steroids (ILS) and autologous platelet-rich plasma (PRP) treatment of oral erosions of PV. In this split-mouth randomized double blind study, one side of the buccal mucosa of 11 PV patients was injected with 10 mg/ml triamcinolone and the opposite side was injected with 1 millilitre of autologous PRP every 14 days for 3 months. The percentage of clinical improvement, pemphigus disease area index and pain score were recorded before and after therapy...
March 10, 2018: Archives of Dermatological Research
Khalaf Kridin, Shira Zelber-Sagi, Reuven Bergman
Although pemphigus is associated with increased mortality, little is known regarding factors that influence prognosis. To identify prognostic factors for mortality at five- and 10-year periods after an initial diagnosis of pemphigus. A retrospective cohort study was performed. Data were collected for all patients with a new diagnosis of pemphigus between 1990 and 2011, who were actively followed for at least five years at our centre. The endpoint was overall survival at five and 10 years after pemphigus diagnosis...
February 1, 2018: European Journal of Dermatology: EJD
Michael Kasperkiewicz, Enno Schmidt, Ralf J Ludwig, Detlef Zillikens
One major hallmark of atopic dermatitis (AD) is the elevated level of total serum IgE, which has been reported to be partly of the autoreactive type in a subset of patients. Immunoadsorption (IA) has been successfully applied in various classical autoantibody-mediated diseases such as pemphigus. Recent reports proposed the use of IA also for patients with severe AD and high total serum IgE levels. In this mini-review, we summarize the current knowledge about this novel treatment approach for AD and briefly discuss the so far incompletely known role of autoreactive IgE as potential target of IA therapy in this common inflammatory skin disorder...
2018: Frontiers in Immunology
Aniek Lamberts, H Ilona Euverman, Jorrit B Terra, Marcel F Jonkman, Barbara Horváth
Introduction: Rituximab (RTX) is a monoclonal antibody targeting CD20, a transmembrane protein expressed on B cells, causing B cell depletion. RTX has shown great efficacy in studies of pemphigus vulgaris, but data of pemphigoid diseases are limited. Objective: To assess the effectiveness and safety of RTX in pemphigoid diseases. Methods: The medical records of 28 patients with pemphigoid diseases that were treated with RTX were reviewed retrospectively...
2018: Frontiers in Immunology
Luca Giannetti, Luigi Generali, Carlo Bertoldi
Involvement of the oral mucosa in patients affected by Pemphigus Vulgaris (PV), Paraneoplastic, IgA Pemphigus and in some cases of Iatrogenic Pemphigus is common, and precede skin lesions in the majority of cases. Intraepidermal bullae are caused by acantholysis, induced by IgG autoantibodies directed against the desmosomes and the domain of numerous keratinocytes self-antigens desmogleins (namely cadherins), thus supporting the autoimmune nature of the disease. Apoptosis may contribute to the acantholysis...
March 6, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Khalaf Kridin, Doron Comaneshter, Erez Batat, Arnon D Cohen
BACKGROUND: Recent evidence indicates that autoimmunity may contribute to the pathogenesis of chronic obstructive pulmonary disease (COPD). COPD was observed at higher frequency in patients with several autoimmune diseases. The association between pemphigus and COPD has not been evaluated in the past. OBJECTIVES: To study the association between pemphigus and COPD using a large-scale real-life computerized database. METHODS: A cross-sectional study was conducted comparing pemphigus patients with age-, sex- and ethnicity-matched control subjects regarding the prevalence of COPD and lung cancer...
March 2018: Respiratory Medicine
D Miyamoto, C W Maruta, C G Santi, P Zoroquiain, A B T Dias, J J Mansure, M N Burnier, V Aoki
BACKGROUND: Erythroderma is a severe manifestation of pemphigus foliaceus (PF), a blistering disease mediated by IgG autoantibodies against desmoglein-1. Increasing evidence supports the contribution of angiogenic mediators in the pathogenesis of erythroderma. OBJECTIVE: To evaluate the in situ expression of vascular endothelial growth factor (VEGF) and endoglin in PF patients with erythroderma. METHODS: Formalin-fixed paraffin-embedded skin samples obtained from patients with erythrodermic PF (n=19; 12 patients with endemic PF), non-erythrodermic PF (n=17), pemphigus vulgaris (PV; n=10), psoriasis (n=10), and healthy individuals (HI; n=10) were processed in an automated immunohistochemistry platform utilizing anti-VEGF and anti-endoglin as primary antibodies...
February 28, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Anastasiya Atanasova Chokoeva, Ilia Lozev, Irina Yungareva, Uwe Wollina, Torello Lotti, Georgi Konstantinov Maximov, Georgi Tchernev
It is well-established that drugs could be leading cause of occurrence of numerous diseases, including pemphigus, being either inducer or triggering factor of the autoimmunity. Despite medications, it should be kept in mind that chronic or acute infections are also capable of being a trigger in various types of cutaneous eruptions, including pemphigus. The rapidly obtained and uncompleted history for accompanied medication leads to general mistakes in the subsequent treatment approach, as the first step in such cases is discontinuation of the drug-inductor...
January 25, 2018: Open Access Macedonian Journal of Medical Sciences
Inga Koneczny
IgG4 autoimmune diseases are characterized by the presence of antigen-specific autoantibodies of the IgG4 subclass and contain well-characterized diseases such as muscle-specific kinase myasthenia gravis, pemphigus, and thrombotic thrombocytopenic purpura. In recent years, several new diseases were identified, and by now 14 antigens targeted by IgG4 autoantibodies have been described. The IgG4 subclass is considered immunologically inert and functionally monovalent due to structural differences compared to other IgG subclasses...
2018: Frontiers in Immunology
Khalaf Kridin
Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. It is characterized by the production of pathogenic autoantibodies directed against different proteins of the desmosome, leading histologically to intraepidermal cleavage, and clinically to vesicles and erosions on the epithelium of the mucous membranes and/or the skin. The diagnosis of the subtype of pemphigus is based on clinical features, the level of histologic cleavage, and the identification of the antigens recognized by circulating autoantibodies by immunoserological analyses...
February 26, 2018: Immunologic Research
O Lai, A Recke, D Zillikens, M Kasperkiewicz
Epidemiological evidence suggests that smoking cigarettes may be beneficial in pemphigus, but no systematic evaluation exists to corroborate this assumption. Therefore, a systematic literature review with pooled data analysis of the smoking status in pemphigus patients was conducted. Electronic searches using PubMed from inception to November 2017 identified 13 reports meeting predetermined inclusion and exclusion criteria. Most were case-control studies partly reporting that pemphigus vulgaris and foliaceus occurred less frequently in current and former smokers...
February 25, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
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