Search results for "Pemphigus"
showing 10 items of 29 documents
Cyclophosphamide plus vincristine and prednisone in the treatment of severe pemphigus vulgaris refractory to conventional therapy
1992
Five patients with severe pemphigus vulgaris refractory to conventional therapy with azathioprine and corticosteroids were treated with cyclophosphamide, vincristine and prednisone. One patient was not evaluable, while the remaining four patients showed a complete response. Duration of response was in the range of 13-94 months. Toxicity was mainly represented by alopecia, myelosuppression and gastrointestinal side-effects such as nausea/vomiting. Although cyclophosphamide and vincristine may induce severe side-effects, this association may be useful in controlling severe disease resistant to previous conventional therapies.
Enteric-coated mycophenolate sodium in the treatment of refractory pemphigus.
2010
BACKGROUND: One of the major goals of pemphigus therapy is to reduce the patient's cumulative exposure to systemic corticosteroids. To investigate the efficacy of enteric-coated mycophenolate sodium (EC-MPS), 10 patients with active, refractory pemphigus vulgaris (PV) or foliaceous (PF) were treated with EC-MPS (1440 mg daily) and prednisone (75 mg daily) over 18 months. OBSERVATIONS: Following EC-MPS/prednisone therapy, disease progression was inhibited between days 30 and 45 in 9/10 patients (8 PV; 1 PF). At 18 months, 8/9 PV patients had clinically quiescent disease; EC-MPS therapy was no longer required in two patients as a result of disease remission. The remaining PV patient showed no…
Epidemiology of desquamative gingivitis: evaluation of 125 patients and review of the literature
2009
Background Desquamative gingivitis (DG) is a descriptive term used to indicate epithelial desquamation, erythema, erosions, and/or vesiculobullous lesions of the gingiva. DG is commonly associated with several mucocutaneous disorders and systemic conditions that may carry a poor prognosis and high morbidity; however, there are no clear data concerning the frequency of these disease associations. Methods We investigated the epidemiologic features of DG in 125 patients and compared our findings with information from a literature review. Results In our series, 88% of patients with DG had one of the following three disorders: oral lichen planus (OLP), mucous membrane pemphigoid (MMP), or pem…
Mycophenolate is effective in the treatment of pemphigus vulgaris.
1999
Background Pemphigus vulgaris is a potentially life-threatening autoimmune disease. Although combination therapies with prednisone and azathioprine are usually effective in controlling the disease, some patients either do not respond to this treatment or show early relapses. Objective To find out whether mycophenolate mofetil would be an effective drug in controlling pemphigus vulgaris in patients who failed initial treatment with azathioprine and prednisone. Results Twelve patients who were initially diagnosed as having pemphigus vulgaris and had relapsed while undergoing treatment with azathioprine (1.5-2 mg/kg of body weight) and prednisolone (2 mg/kg of body weight) subsequently receive…
Effects of metformin on autoimmune immunoglobins and interferon-γ in patients with early diagnosed pemphigus vulgaris: a prospective clinical trial.
2021
The management of pemphigus vulgaris (PV) is challenging. This study aimed to evaluate the immunomodulating effects of metformin on PV. The study was conducted in two phases: in the first phase, patients received routine first-line treatment (prednisolone plus azathioprine) for 2 months, then in the second phase, metformin was added to this regimen for another 2 months. After addition of metformin to the first-line medications, significant reductions were seen in serum IgG1 (reduced from 534.92 ± 134.83 mg/dL to 481.58 ± 130.46 mg/dL, P < 0.001), IgG4 (51.83 ± 27.26 mg/dL to 44.50 ± 26.05 mg/dL, P < 0.001) and interferon-γ (277.99 ± 108.71 pg/mL to 45.05 ± 17.080 pg/mL, P = 0.03) concentrat…
Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID)
2011
Introduction: Evidence from a number of open-label, uncontrolled studies has suggested that rituximab may benefit patients with autoimmune diseases who are refractory to standard-of-care. The objective of this study was to evaluate the safety and clinical outcomes of rituximab in several standard-of-care-refractory autoimmune diseases (within rheumatology, nephrology, dermatology and neurology) other than rheumatoid arthritis or non-Hodgkin's lymphoma in a real-life clinical setting. Methods: Patients who received rituximab having shown an inadequate response to standard-of-care had their safety and clinical outcomes data retrospectively analysed as part of the German Registry of Autoimmune…
Rituximab in refractory pemphigus vulgaris
2008
Pemphigus vulgaris (PV) is a severe chronic autoimmune blistering disease of skin and mucous membranes. The use of systemic corticosteroids in pemphigus has dramatically reduced its mortality rate, but the long-term use of steroids leads to severe side effects, many of which are serious. For this reason it is often necessary to add immunosuppressive agents to the regimen. However, there are occasional refractory cases in which therapy with conventionally accepted modalities is either not efficacious or not possible on account of side effects. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and a…
Molecular characterization of the leucine cluster in Buchnera PSY, primary endosymbiont of the aphid Pemphigus spyrothecae
2002
ABSTRACT Buchnera strains from most aphid subfamilies studied to date have been found to carry the leucine gene cluster ( leuA , - B , - C , and - D ) on a plasmid, an organization unique among bacteria. Here, however, we demonstrate a classical chromosomal location of the cluster in Buchnera sp. strain PSY from the aphid Pemphigus spyrothecae (subfamily Pemphiginae). The genes that flank leuABCD in Buchnera sp. strain PSY appear to be adjacent in the genome of Buchnera sp. strain APS, a strain carrying a leucine plasmid. We propose that the presence of a leucine plasmid predates the diversification of symbiotic Buchnera and that the chromosomal location observed in Buchnera sp. strain PSY …
Diagnostic pathways and clinical significance of desquamative gingivitis:a review
2008
The term desquamative gingivitis (DG) refers to a clinical manifestation that can be caused by several disorders. Many of them are immunologically mediated; in addition to the oral cavity, they can affect extraoral mucocutaneous sites, e.g., larynx, conjunctiva, esophagus, nasal and genital mucosa, and the skin. The degree of oral, periodontal, and systemic involvement determines the overall morbidity and, sometimes, the mortality of these disorders. We comprehensively review disorders commonly associated with DG and highlight diagnostic pathways, guidelines for differential diagnosis, and oral, periodontal, and systemic implications. More rare conditions are reviewed as well. Mucous membra…
Langerhans Cells Prevent Autoimmunity via Expansion of Keratinocyte Antigen-Specific Regulatory T Cells
2017
Langerhans cells (LCs) are antigen-presenting cells in the epidermis whose roles in antigen-specific immune regulation remain incompletely understood. Desmoglein 3 (Dsg3) is a keratinocyte cell-cell adhesion molecule critical for epidermal integrity and an autoantigen in the autoimmune blistering disease pemphigus. Although antibody-mediated disease mechanisms in pemphigus are extensively characterized, the T cell aspect of this autoimmune disease still remains poorly understood. Herein, we utilized a mouse model of CD4+ T cell-mediated autoimmunity against Dsg3 to show that acquisition of Dsg3 and subsequent presentation to T cells by LCs depended on the C-type lectin langerin. The lack of…