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Paraneoplastic Pemphigus

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181. Pemphigus Full Text available with Trip Pro

bullae are subepidermal. Three major variants of the disorder have been described, each with characteristic clinical and immunological features: Pemphigus vulgaris (PV) is the most common subset or variant and accounts for 70% of cases of pemphigus. Pemphigus foliaceus (PF) is characterised by lesions which occur only in the skin and associated with antibodies to desmoglein 1 (DSG1). Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms (...) lesions. Pemphigus foliaceus (PF): Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. Paraneoplastic pemphigus (PNP): It may present in patients who are already known to have an underlying neoplasm such as the B-cell lymphoproliferative disorders (eg, non-Hodgkin's lymphoma and chronic lymphocytic leukaemia). It may present with mucocutaneous lesions before any underlying tumour has been diagnosed. Patients present with painful ulcers on their mucous

2008 Mentor

182. Phase II Study of High-Dose Cyclophosphamide in Patients With Refractory Pemphigus

activity despite treatment with mycophenolate mofetil or azathioprine, or inability to tolerate these drugs (for paraneoplastic pemphigus, persistent disease activity despite treatment with cyclosporine) --Prior/Concurrent Therapy-- No concurrent cytotoxic therapy --Patient Characteristics-- Performance status: Karnofsky 20-100% Renal: Creatinine no greater than 2.5 mg/dL Cardiovascular: LVEF at least 40% Pulmonary: FVC, FEV1, or DLCO at least 50% predicted Other: Not pregnant Fertile patients must use (...) Phase II Study of High-Dose Cyclophosphamide in Patients With Refractory Pemphigus Phase II Study of High-Dose Cyclophosphamide in Patients With Refractory Pemphigus - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before

2001 Clinical Trials

183. A unique form of endemic pemphigus in northern Colombia. (Abstract)

pemphigus. It shares some heterogeneous immunoreactivity with paraneoplastic pemphigus but is not associated with malignant tumors. The disease resembles Senear-Usher syndrome (pemphigus and lupus) but occurs endemically either with a localized stable clinical course or in a systemic form. This systemic form may affect organs other than skin and is characterized by episodic relapses and poor prognosis in comparison with the localized form.We have confirmed endemic pemphigus foliaceus in El Bagre (...) as an autoimmune disease that shares features with Senear-Usher syndrome but occurs in an endemic fashion. Heterogeneous antigenic reactivity is observed as in paraneoplastic pemphigus but with no evidence of association with neoplasia. In addition, constant exogenous antigenic stimulation and a genetic predisposition may be required in the pathogenesis of this disease.

2003 Journal of American Academy of Dermatology

184. Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. (Abstract)

proteins with molecular weights of 250 kd, 210 kd, and 190 kd, which appear to correspond to desmoplakin I, envoplakin, and periplakin, respectively.This endemic pemphigus disease in El Bagre showed immunologic features similar to pemphigus foliaceus or erythematosus. In addition, paraneoplastic pemphigus-like reactivity with various epidermal antigens was detected. (...) Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. We previously described a new focus of endemic pemphigus foliaceus in rural areas of El Bagre, Colombia, with clinical and direct immunofluorescence characteristics of pemphigus erythematosus.The aim of this study was to characterize autoantigen profiles for 34 serum samples obtained from patients with this condition.Immunofluorescence, various immunoblot analyses with different antigen sources and detection

2003 Journal of American Academy of Dermatology

185. An evaluation of the usefulness of mycophenolate mofetil in pemphigus. (Abstract)

pemphigus.Patients with active, refractory pemphigus were treated with MMF. Our series included 12 cases of pemphigus vulgaris, four cases of pemphigus foliaceous and one case of paraneoplastic pemphigus. All patients were monitored to assess disease control and mycophenolate toxicity.Of the 17 cases, MMF has been of benefit to 12. MMF was well tolerated and there were no treatment withdrawals because of safety concerns.We found that MMF permitted a reduction in prednisolone dosage without disease relapse. (...) An evaluation of the usefulness of mycophenolate mofetil in pemphigus. Pemphigus is a group of autoimmune blistering diseases of the skin and/or mucous membranes requiring management with immunosuppressive therapy. The optimal therapeutic regimen would rapidly induce remission and maintain effectiveness with minimal adverse effects in the long term.The present study describes our experience of the addition of mycophenolate mofetil (MMF) to prednisolone in the management of severe, refractory

2003 British Journal of Dermatology

186. Castleman's tumours and production of autoantibody in paraneoplastic pemphigus. (Abstract)

Castleman's tumours and production of autoantibody in paraneoplastic pemphigus. Paraneoplastic pemphigus is an autoimmune mucocutaneous disease associated with Castleman's tumours, which when surgically removed often result in great improvement of mucocutaneous lesions. An IgG autoantibody against epidermal proteins is often used as a diagnostic marker for disease. Our aim was to ascertain the role of Castleman's tumours in production of the autoantibody and pathogenesis of paraneoplastic (...) pemphigus.We enrolled seven patients with paraneoplastic pemphigus associated with Castleman's disease and assessed the effect of removal of tumours on mucocutaneous lesions in six individuals and on autoantibody titre with indirect immunofluorescence in four patients. We cultured tumour cells from one patient and assayed the secreted autoantibody. Finally, we characterised the gene sequence and expression of the variable region of the immunoglobulin heavy chain (IgV(H)) in tumour B cells from all patients

2004 Lancet

187. Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature Full Text available with Trip Pro

Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature Castleman's disease is a rare lymphoproliferative syndrome. Its etiology and pathogenesis are unclear. The disease can be occasionally associated with a paraneoplastic pemphigus (PNP), an autoimmune mucocutaneous disorder commonly seen in neoplasms of lymphocytic origin.We present a case of a 63-year old male patient who was referred for surgical treatment of a lately diagnosed (...) retroperitoneal pelvic mass. The patient had been already treated for two years due to progressive diffuse cutaneous lesions histologically consistent with lichen ruber verucosus and pemphigus vulgaris. Intraoperatively a highly vascularized solid mass occupying the small pelvis was resected after meticulous vascular ligation and hemostasis. After surgery and following immunosuppressive treatment a clear remission of the skin lesions was observed.Castleman's tumor should be always suspected when

2007 World journal of surgical oncology

188. Anaplastic large cell lymphoma presenting with paraneoplastic pemphigus Full Text available with Trip Pro

Anaplastic large cell lymphoma presenting with paraneoplastic pemphigus 17213360 2007 02 20 2018 11 13 0021-9746 60 1 2007 Jan Journal of clinical pathology J. Clin. Pathol. Anaplastic large cell lymphoma presenting with paraneoplastic pemphigus. 108-10 Davis A K AK Cole-Sinclair M M Russell P P eng Case Reports Letter England J Clin Pathol 0376601 0021-9746 AIM IM Adult Autoimmune Diseases etiology pathology Fatal Outcome Female Humans Lymphoma, Large-Cell, Anaplastic complications diagnosis (...) Multiple Organ Failure etiology Paraneoplastic Syndromes etiology pathology Pemphigus etiology pathology 2007 1 11 9 0 2007 2 21 9 0 2007 1 11 9 0 ppublish 17213360 60/1/108-a 10.1136/jcp.2005.036137 PMC1860589 Arch Dermatol. 2001 Feb;137(2):193-206 11176692 N Engl J Med. 1999 May 6;340(18):1406-10 10228191 N Engl J Med. 1990 Dec 20;323(25):1729-35 2247105 Br J Dermatol. 2001 Jun;144(6):1102-4 11422027

2007 Journal of Clinical Pathology

189. Epitopes in the linker subdomain region of envoplakin recognized by autoantibodies in paraneoplastic pemphigus patients. Full Text available with Trip Pro

Epitopes in the linker subdomain region of envoplakin recognized by autoantibodies in paraneoplastic pemphigus patients. Sera from paraneoplastic pemphigus (PNP) immunoprecipitate multiple antigens from human epidermal protein extract. In this study, we further characterized the autoantibodies in 12 PNP sera. Immunoblotting using recombinant linker subdomains of envoplakin, periplakin, desmoplakin, and bullous pemphigoid antigen I found that 11 of the 12 sera recognized linker subdomains

2006 Journal of Investigative Dermatology

190. Genotyping of HLA-I and HLA-II alleles in Chinese patients with paraneoplastic pemphigus. (Abstract)

Genotyping of HLA-I and HLA-II alleles in Chinese patients with paraneoplastic pemphigus. Class I and class II HLA genes are thought to play a role in the immunopathogenesis of bullous dermatoses such as pemphigus vulgaris and pemphigus foliaceus, but we know little about the genetic background of paraneoplastic pemphigus (PNP) in Chinese patients.To identify class I and class II HLA alleles by genotyping in Chinese patients with PNP, and to find out the possible association between HLA alleles (...) , and the other alleles were unremarkable in these patients.The genetic background predisposing to PNP may be different in patients from various races and areas. HLA-Cw*14 may be the predisposing allele to PNP in Chinese patients, which is different from the predisposing allele in French patients with PNP and the alleles predisposing to pemphigus vulgaris and pemphigus foliaceus.

2007 British Journal of Dermatology

191. Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies. (Abstract)

Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies. Paraneoplastic pemphigus (PNP) has been described as an antibody-mediated mucocutaneous disease occurring almost exclusively in patients with lymphocytic neoplasms. We describe 4 patients with the clinical features of the lichenoid variant of PNP in the absence of detectable autoantibodies. On the basis of these findings, we conclude that the spectrum of PNP likely includes patients with disease predominantly

2007 Journal of American Academy of Dermatology

192. A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia. (Abstract)

A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia. Paraneoplastic pemphigus is an autoimmune vesiculobullous and erosive mucocutaneous disease associated with an underlying malignancy. Reported malignancies include chronic lymphocytic leukemia, non-Hodgkin's lymphoma, Castleman's disease, sarcomas, and rarely solid tumors. Patients with paraneoplastic pemphigus develop characteristic IgG autoantibodies against several antigens including members (...) of the plakin family, bullous pemphigoid antigen 1, and desmosomal proteins. IgA pemphigus is another recently characterized immunobullous disease that presents as a vesiculopustular eruption with neutrophilic infiltration and epidermal acantholysis. Mucous membrane involvement is rare. We report what is to our knowledge a unique case with features of both IgA pemphigus and paraneoplastic pemphigus associated with chronic lymphocytic leukemia.

2007 Journal of American Academy of Dermatology

193. Progressive respiratory failure in paraneoplastic pemphigus associated with chronic lymphocytic leukemia. (Abstract)

Progressive respiratory failure in paraneoplastic pemphigus associated with chronic lymphocytic leukemia. A 70-year-old Caucasian man with chronic lymphocytic leukemia suffered from widespread, histologically proven cutaneous lichen planus responding to topical corticosteroids. 2 years later, he presented with painful erosive stomatitis and increasing dyspnea. Histology, direct and indirect immunofluorescence were diagnostic for paraneoplastic pemphigus. A full diagnostic workup could (...) not disclose the cause of the progressive respiratory insufficiency. Despite aggressive treatment of the lymphocytic leukemia and the paraneoplastic pemphigus, the patient died 3 months after diagnosis. Paraneoplastic pemphigus may lead to pulmonary failure which is refractory to treatment and has a fatal outcome.Copyright 2004 S. Karger AG, Basel

2004 Dermatology

194. Paraneoplastic pemphigus with clinical features of lichen planus associated with low-grade B cell lymphoma. Full Text available with Trip Pro

Paraneoplastic pemphigus with clinical features of lichen planus associated with low-grade B cell lymphoma. Neoplasia-induced lichen planus is described as a cell-mediated reaction to unknown epithelial antigens. Paraneoplastic pemphigus (PNP), characterized by the presence of a specific array of autoantibodies, probably represents a different form of presentation of the same autoimmune syndrome where the mucocutaneous expression depends on the dominant pathologic mechanism.The authors report

2005 International Journal of Dermatology

195. Paraneoplastic pemphigus resembling linear IgA bullous dermatosis. (Abstract)

Paraneoplastic pemphigus resembling linear IgA bullous dermatosis. A 69-year-old Chinese man presented in 2001 with a blistering eruption over the upper and lower limbs associated with oral ulceration for 1 month. He had stage IIIA follicular small cell cleaved non-Hodgkin's lymphoma diagnosed 5 years previously, and had received several lines of palliative chemotherapy, including two courses of chlorambucil, six cycles of cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP (...) -intercellular antibody at a titre of 1/160 and positive on rat bladder at a titre of 1/80. A presumptive diagnosis of paraneoplastic pemphigus was made. This was later confirmed by the presence of antibodies against envoplakin (210 kDa), periplakin (190 kDa), and desmoglein 1 on immunoprecipitation studies. He was started on prednisolone 60 mg/day (1 mg/kg/day), with complete resolution of skin lesions within 1 week, but persistence of oral ulcers. Cyclophosphamide was added at a low dose of 1 mg/kg/day

2006 International Journal of Dermatology

196. Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies. (Abstract)

Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies. A 48-year-old woman with a follicular, grade III, B-cell non-Hodgkin lymphoma developed clinical, immunopathological and histological features of paraneoplastic pemphigus. The skin symptoms flared after repeated cyclophosphamide infusions, and were preceded and accompanied by a progressive dyspnoea. Although the skin and oral mucosal disease went into remission (...) serum contained IgG and IgA antibodies to desmocollin 3 as well as IgG antibodies to desmocollin 2. Serum IgG and IgA strongly stained rat bronchial epithelium, corresponding to autoantibodies possibly involved in the pathomechanism of the severe lung disease. In this case, which was characterized by a mixed IgA/IgG antibody panel displaying known and unique antigenicity, the serious episodes of paraneoplastic pemphigus flared after cyclophosphamide treatment.

2004 British Journal of Dermatology

197. Use of biosynthetic dressings in paraneoplastic pemphigus. (Abstract)

Use of biosynthetic dressings in paraneoplastic pemphigus. A 77-year-old woman with paraneoplastic pemphigus and non-Hodgkin's lymphoma was treated with supportive therapy and oral prednisone. Biobrane, a biosynthetic dressing, was later applied to the extensive areas of erosion to assist in pain management and to provide a temporary barrier function. She reported an improvement in the pain associated with the areas of erosion. The use of biosynthetic dressings in blistering disorders has

2004 Australasian Journal of Dermatology

198. Paraneoplastic pemphigus: a brief update. (Abstract)

Paraneoplastic pemphigus: a brief update. First described in 1990, paraneoplastic pemphigus is an autoimmune condition that causes considerable morbidity, is resistant to therapy and is frequently fatal. Clinical heterogeneity is being recognized as more cases are reported and the documented auto-antigen profile is also increasing. Target antigens are now known to be not restricted to the skin, suggesting that this condition is part of a paraneoplastic autoimmune multiorgan syndrome.

2005 Australasian Journal of Dermatology

199. Antibody titers to desmogleins 1 and 3 in a patient with paraneoplastic pemphigus associated with follicular dendritic cell sarcoma. Full Text available with Trip Pro

Antibody titers to desmogleins 1 and 3 in a patient with paraneoplastic pemphigus associated with follicular dendritic cell sarcoma. Most paraneoplastic pemphigus (PNP) cases reported to date have been associated with lymphoproliferative neoplasms. Patients with PNP have autoantibodies against the plakin family (eg, envoplakin and periplakin). Antibodies against desmoglein 3 (Dsg3) and Dsg1, antigens for classic types of pemphigus, have also been reported to play an important role (...) the clinical phenotype and the anti-Dsg antibody profile, as seen in pemphigus vulgaris.

2004 Archives of Dermatology

200. Paraneoplastic pemphigus associated with non-Hodgkin B-cell lymphoma and good response to prednisone. Full Text available with Trip Pro

Paraneoplastic pemphigus associated with non-Hodgkin B-cell lymphoma and good response to prednisone. Paraneoplastic pemphigus is a life-threatening autoimmune bullous disease associated with neoplasia, generally of lymphoid origin. Immunosuppressive therapy is often disappointing and there are only a few reports of patients surviving more than 2 years. These cases were generally associated with benign neoplasms. We report here the case of a patient with paraneoplastic pemphigus associated

2005 Acta Dermato-Venereologica

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