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Paraneoplastic Pemphigus

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161. Retroperitoneal liposarcoma associated with small plaque parapsoriasis (PubMed)

Retroperitoneal liposarcoma associated with small plaque parapsoriasis Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis (...) syndrome was done.Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma.

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2007 World journal of surgical oncology

162. Lower But More Frequent Dose Rituximab to Treat Chronic Lymphocytic Leukemia

to tolerate protocol therapy History of mucocutaneous reactions (paraneoplastic pemphigus, Stevens-Johnson syndrome, lichenoid dermatitis, vesiculobullous dermatitis, and toxic epidermal necrolysis) Known anaphylaxis or IgE mediated hypersensitivity to murine proteins or to any component of this product Inability to self inject the study medication or to have it administered by a third person Inability to understand the investigational nature of the study ability to provide informed consent Contacts

2006 Clinical Trials

163. Castleman's disease associated with myasthenia gravis. (PubMed)

, organomegally, endocrinopathy, monoclonal gammopathy, and skin changes), paraneoplastic pemphigus, Hodgkin's disease, and follicular dendritic cell sarcoma. We present a case of Castleman's disease associated with myasthenia gravis, the third reported case in the literature. We discuss Castleman's disease and review the literature.

2003 Annals of Thoracic Surgery

164. Management of autoimmune skin disorders in the elderly. (PubMed)

therapy, high-potency topical corticosteroids are very useful as initial treatment and, in the elderly, should be used instead of oral prednisolone wherever possible. Pemphigus is a chronic blistering disease of which there are two main subtypes: vulgaris and foliaceous. Paraneoplastic pemphigus is a unique clinical, histological and immunologically distinct autoimmune mucocutaneous disease which tends to be relentlessly progressive. Lichen sclerosus presents specific complications and a small

2004 Drugs & Aging

165. Rituximab in refractory autoimmune bullous diseases. (PubMed)

in 26 treatment-resistant patients with the vulgaris, foliaceus, and paraneoplastic variants of pemphigus as well as in bullous pemphigoid and epidermolysis bullosa acquisita. All but a single patient showed clinical improvement with reduction of lesion formation. In about a third, a clinical remission requiring further immunsuppressive medication was achieved, and in about a quarter, complete remission was induced. In addition, the mode of action and adverse events of rituximab as well as adjuvant

2006 Clinical & Experimental Dermatology

166. PNP with Waldenström's macroglobulinaemia. (PubMed)

PNP with Waldenström's macroglobulinaemia. We describe a 65-year-old Caucasian man with Waldenstrom's macroglobulinaemia who developed paraneoplastic pemphigus (PNP) 3 years after his haematological diagnosis. This is a very rare malignancy that is associated with PNP. The evolution of PNP in this patient appears to exhibit the postulated immunological phenomenon of epitope spreading.

2005 Clinical & Experimental Dermatology

167. Sera from patients with toxic epidermal necrolysis contain autoantibodies to periplakin. (PubMed)

Sera from patients with toxic epidermal necrolysis contain autoantibodies to periplakin. The pathophysiological mechanism of toxic epidermal necrolysis (TEN) with extensive bullae that is induced suddenly by drugs is not well understood. The individual patterns and distribution of the widespread mucocutaneous reactions of TEN often show striking similarities with those of paraneoplastic pemphigus (PNP), which is known to involve autoantibodies (aAbs) to members of the plakin family.To

2006 British Journal of Dermatology

168. Rituximab: applications in dermatology. (PubMed)

Rituximab: applications in dermatology. Rituximab is a chimeric anti-CD20 monoclonal antibody which has been used extensively for B-lymphocytic malignancies. In addition, applications for autoimmune diseases have emerged in recent years. Case reports support the use of rituximab in certain dermatologic conditions, including paraneoplastic pemphigus, pemphigus vulgaris, graft versus host disease, and cutaneous B-cell malignancies. Clinical trials are lacking and would be an appropriate next step.

2006 International Journal of Dermatology

169. Human placental amnion is a novel substrate for detecting autoantibodies in autoimmune bullous diseases by immunoblotting. (PubMed)

to desmosomal and BMZ proteins, and with sera from 76 patients with AIBDs including pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid (BP), pemphigoid gestationis, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, paraneoplastic pemphigus and mucous membrane pemphigoid.The desmosomes and BMZ of the amnion tissue were ultrastructurally similar to those in skin. Antigen mapping confirmed that amnion contains all the proteins that were recognized by a panel of monoclonal and polyclonal

2003 British Journal of Dermatology

170. A unique form of endemic pemphigus in northern Colombia. (PubMed)

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

171. Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. (PubMed)

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

172. Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies. (PubMed)

Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies. Anti-desmoplakin (DP) antibodies are present in paraneoplastic pemphigus (PNP) as a component of a complex humoral autoimmune reaction characterized by antibodies against proteins of the plakin family, desmogleins, and an unidentified 170 kd protein. Anti-DP antibodies have also been rarely identified in other blistering diseases. The significance of anti-DP antibodies in the pathogenesis of bullous diseases is unclear.We (...) studied 3 patients with severe and chronic mucosal dominant pemphigus vulgaris (PV). In addition to anti-desmoglein 3 antibodies, these patients had anti-DP autoantibodies, demonstrable by immunofluorescence (IF), immunoprecipitation (IP), and indirect immunoelectromicroscopy (IIEM). This finding suggested these patients may have had PNP and not PV. However, antibodies against periplakin, envoplakin, bullous pemphigoid antigen 1 (BPAG 1), plectin, and 170 kd PNP antigen could not be detected using IP

2004 Journal of American Academy of Dermatology

173. Antidesmoplakin antibodies in pemphigus vulgaris. (PubMed)

Antidesmoplakin antibodies in pemphigus vulgaris. Besides being present in paraneoplastic pemphigus (PNP), circulating antidesmoplakin (DP) antibodies have been found anecdotally in other bullous diseases, including pemphigus foliaceus and pemphigus vulgaris.To verify how frequent anti-DP antibodies are in pemphigus vulgaris.We studied 48 sera from patients with proven pemphigus vulgaris (29 mucosal dominant pemphigus and 19 mucocutaneous pemphigus) by indirect immunofluorescence (IIF) with rat (...) , which, in patients with pemphigus vulgaris, are probably caused by an epitope-spreading phenomenon.

2006 British Journal of Dermatology

174. An evaluation of the usefulness of mycophenolate mofetil in pemphigus. (PubMed)

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

175. A case of pemphigus vulgaris accompanied by multiple myeloma. (PubMed)

myeloma. From the results of immunoblotting using normal human epidermal extracts and indirect immunofluorescence using rat bladder sections, and her clinical manifestations, our case does not seem to be one of paraneoplastic pemphigus. (...) A case of pemphigus vulgaris accompanied by multiple myeloma. Pemphigus is a mucocutaneous intraepithelial blistering disease caused by autoantibodies to epithelial cell adhesion molecules (desmoglein). The association between pemphigus and malignant neoplasm is well recognized. We present the case of a 62-year-old woman with pemphigus vulgaris accompanied by multiple myeloma. To the best of our knowledge, this is the first report of a case of pemphigus vulgaris concomitant with multiple

2005 International Journal of Dermatology

176. 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

177. Castleman's tumours and production of autoantibody in paraneoplastic pemphigus. (PubMed)

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

178. Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature (PubMed)

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

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2007 World journal of surgical oncology

179. Anaplastic large cell lymphoma presenting with paraneoplastic pemphigus (PubMed)

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

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2007 Journal of Clinical Pathology

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

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

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