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pemphigus vulgaris

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
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
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
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
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
Elizabeth Cusick, David Silverstein
Although significant progress has been made for the treatment of pemphigus vulgaris (PV) with rituximab (RTX), a consensus remains to be determined for standard treatment protocol, regarding optimal dosing, infusion regimen, and use of concomitant immunotherapy to achieve safe, effective, and rapid clinical response. We describe a patient with pemphigus vulgaris treated with high dose rituximab with the rheumatoid arthritis protocol along with intravenous immunoglobulin therapy. This case provides evidence towards the growing body of research needed to modify and improve treatment for pemphigus using rituximab...
September 15, 2017: Dermatology Online Journal
Khalaf Kridin, Shira Zelber-Sagi, Doron Comaneshter, Arnon D Cohen
Importance: The association of pemphigus vulgaris and pemphigus foliaceus with comorbid malignancies is yet to be firmly established. Objective: To estimate the association between pemphigus and a wide range of nonhematologic malignancies using one of the largest cohorts of patients with pemphigus to date. Design, Setting, and Participants: For this cross-sectional study, we used the computerized database of Clalit Health Services, the largest public health care provider organization in Israel insuring 4...
February 16, 2018: JAMA Dermatology
Ashley E Brown, Kiran Motaparthi, Sylvia Hsu
Rituximab and intravenous immunoglobulin [IVIg] have recently emerged as effective treatments for pemphigus refractory to corticosteroids [CS]. This case series sought to compare the clinical, serologic,and adverse effects of CS, IVIg, and rituximab in patients with pemphigus. A retrospective review of 63 patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), or paraneoplastic pemphigus (PNP)was performed. Clinical remission (CR), serologic remission (SR), and adverse effects were evaluated. Three study groups were compared: patients treated with systemic CS, refractory patients treated withIVIg, and refractory patients treated with rituximab...
January 19, 2018: Dermatology Online Journal
Derek Y Hsu, Joaquin Brieva, Beatrice Nardone, Dennis West, Jonathan I Silverberg
BACKGROUND: Pemphigus is a group of debilitating autoimmune blistering disorders associated with painful blisters of the skin and/or mucous membranes. Identification and management of the comorbiditiesof pemphigus is critically important to minimize morbidity and decrease mortality. OBJECTIVE: To identify the comorbid health conditions of pemphigus vulgaris. METHODS: This was a case-control study of 130 cases of pemphigus verified by a clinical and laboratory diagnosis and 390 age and sex-matched controls with complete follow-up at a large metropolitanquaternary care medical center...
December 15, 2017: Dermatology Online Journal
Joo Wan Seo, Jongha Park, Jin Lee, Mi Young Kim, Hyun Ju Choi, Heui Jeong Jeong, Ji Woon Lee, So Young Jung, Woo Kyeong Kim
Pemphigus vulgaris is an autoimmune bullous disorder characterized by the production of autoantibodies against the intercellular space of the epithelium. It has rarely been reported in association with inflammatory bowel disease. Ulcerative colitis is one of the forms of inflammatory bowel disease. A 62-year-old woman who had been treated for ulcerative colitis for 16 years developed pruritic bullae on the skin of her face and body. Histological findings and direct immunofluorescence examination of the skin showed pemphigus vulgaris...
January 2018: Intestinal Research
Marwah Adly Saleh
BACKGROUND: Rituximab (RTX) is an effective therapy for patients with pemphigus. However it does not prevent relapse. OBJECTIVES: To compare early relapsing patients (before 12 months) and late relapsing patients (after 24 months) following RTX therapy. METHODS: In this prospective study, 19 patients were enrolled (14 pemphigus vulgaris and 5 pemphigus foliaceus). The baseline disease score, autoantibodies, and percentage of CD20+ cells of pemphigus patients were measured...
January 30, 2018: Journal of the American Academy of Dermatology
Alexandra C Perks, Paula M Farthing, Ruth Murphy, Anne M Hegarty
Pemphigus is a chronic autoimmune condition that can affect multiple areas of the body. The two main subtypes of pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF) which can rarely occur concurrently or even transition from one to the other. The process of transition may be explained by qualitative changes in desmoglein autoantibody profile. We present a rare case of concomitant PF and oral PV and explore the literature on transitions between pemphigus subtypes and whether this case could represent a transition from PF to PV...
January 16, 2018: Head and Neck Pathology
H Yuan, M Pan
No abstract text is available yet for this article.
December 2017: British Journal of Dermatology
Robert Pollmann, Thomas Schmidt, Rüdiger Eming, Michael Hertl
Pemphigus is a group of rare, potentially devastating autoimmune diseases of the skin and mucous membranes with high morbidity and potentially lethal outcome. The major clinical variant, pemphigus vulgaris (PV) is caused by a loss of intercellular adhesion of epidermal keratinocytes which is induced by IgG autoantibodies against components of desmosomes. Specifically, IgG against the desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1), preferentially target their ectodomains which are presumably critical for the transinteraction and signalling function of these adhesion molecules...
January 8, 2018: Clinical Reviews in Allergy & Immunology
Fatemeh Atarzadeh, Amir Mohammad Jaladat, Babak Daneshfard, Ladan Dastgheib, Mohammad Kamalinejad, Gholamreza Amin
Objective: As a rare autoimmune disease, pemphigus vulgaris has a poor prognosis especially in lack of proper medical support. This blistering disease involves both the skin and mucus membranes. The challenge is improving the healing process of skin lesions of which, superimposed infections are among the main causes of the disease mortality. Accordingly, we aimed to assess the treatment options suggested by traditional Persian medicine (TPM) and compare them with current findings. Materials and Methods: We studied the main clinical and pharmaceutical textbooks of TPM (Kitāb al-hāwīfī al-tibb, the Canon of Medicine, Eksir-e-Aazam, Tuhfat al-mu'minīn, Makhzan al-adviyah (focusing on the skin chapter and respective herbal remedies for the inflamed skin and ulcers...
November 2017: Avicenna Journal of Phytomedicine
M Ormond, H McParland, A N A Donaldson, M Andiappan, R J Cook, M Escudier, E Hullah, J Higham, R McMillan, J Taylor, P J Shirlaw, S J Challacombe, J F Setterfield
OBJECTIVES: The primary aim of this study was to validate the Oral Disease Severity Score (ODSS) for the assessment of oral involvement in pemphigus vulgaris (PV). A secondary aim was to compare the inter - intra- observer variability and ease of use with the Physician's Global Assessment (PGA) and the oral scoring methods used in Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and the Pemphigus Disease Area Index (PDAI). METHODS: 15 patients with mild to moderately severe oral PV were scored for disease severity by 10 oral medicine clinicians using the ODSS, PGA and the oral sections of ABSIS and PDAI...
January 3, 2018: British Journal of Dermatology
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