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213 results for

Paraneoplastic Pemphigus

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201. Paraneoplastic pemphigus without an underlying neoplasm. (Abstract)

Paraneoplastic pemphigus without an underlying neoplasm. We describe a 52-year-old man with paraneoplastic pemphigus (PNP) without any evidence of an underlying neoplasm over an 8-year follow-up period. He had a chronic relapsing vesiculobullous eruption for approximately 7 years (from April 1998 to May 2005). Initially, scattered flaccid vesicles with crusts developed on the face and trunk, which waxed and waned several times. Our patient was diagnosed as having PNP based on immunopathological

2007 British Journal of Dermatology

202. Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. (Abstract)

Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease. We previously reported that B cells in a Castleman tumour associated with PNP produced autoantibodies. However, it is uncertain whether the production of autoantibodies from the associated tumour is a common mechanism in PNP.To investigate autoantibody production in a thymoma and a follicular dendritic

2005 British Journal of Dermatology

203. Paraneoplastic pemphigus associated with CD20-positive follicular non-Hodgkin's lymphoma treated with rituximab: a third case resistant to rituximab therapy. (Abstract)

Paraneoplastic pemphigus associated with CD20-positive follicular non-Hodgkin's lymphoma treated with rituximab: a third case resistant to rituximab therapy. Paraneoplastic pemphigus is an IgG-mediated disease characterized clinically by a polymorphous blistering eruption with severe mucosal involvement associated with an underlying or occult malignancy. It is associated with high mortality, and response to treatment is generally poor. Potent immunosuppression is often necessary to control (...) progression of the disease. Three case reports have documented successful treatment of paraneoplastic pemphigus with rituximab, an anti-CD20 monoclonal antibody. However, two previous reports have noted that rituximab was unsuccessful in halting progression of PNP. We report a third case of paraneoplastic pemphigus associated with follicular non-Hodgkin's lymphoma in which rituximab was not effective. Whether rituximab is an effective and novel treatment for paraneoplastic pemphigus remains undecided.

2007 Clinical & Experimental Dermatology

204. Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) in a child: case report and review of the literature. Full Text available with Trip Pro

Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) in a child: case report and review of the literature. Paraneoplastic autoimmune multiorgan syndrome, also known as paraneoplastic pemphigus, has been observed only rarely among children. We describe a 10-year-old boy with typical clinical and histologic findings of paraneoplastic pemphigus associated with Castleman's disease. His disease was refractory to resection of the tumor and aggressive combination immunosuppressive

2004 Pediatrics

205. Human autoantibodies against desmoplakins in paraneoplastic pemphigus. Full Text available with Trip Pro

Human autoantibodies against desmoplakins in paraneoplastic pemphigus. Recently, a previously unrecognized autoantibody mediated blistering disease, paraneoplastic pemphigus has been described. Paraneoplastic pemphigus is associated with lymphoid malignancies, thymomas, and poorly differentiated sarcomas. Serum of affected patients contain pathogenic autoantibodies that immunoprecipitate from normal keratinocytes a characteristic complex of four polypeptides with M(r) of 250, 230, 210, and 190 (...) to homogeneity from pig tongue epithelium. By indirect immunofluorescence, 9 of 11 sera showed strong binding to epithelial and nonepithelial desmosomes, and 2 were weakly reactive. All 11 immunoprecipitated 250- and 210-kD bands of variable intensity that comigrated with bands identified by a murine monoclonal antidesmoplakin antibody, and immunoblotting confirmed binding of the serum autoantibodies to purified desmoplakins. This demonstrates that paraneoplastic pemphigus is the first human autoimmune

1992 Journal of Clinical Investigation

206. Paraneoplastic pemphigus in association with hepatocellular carcinoma. (Abstract)

Paraneoplastic pemphigus in association with hepatocellular carcinoma. Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous blistering disease associated with neoplasms, most frequently of the lymphoproliferative type. Rare PNP cases related to nonhematological solid tumors have been reported. The patient in this report presented with severe mucocutaneous involvement of PNP associated with hepatocellular carcinoma. Histopathology showed vacuolar interface dermatitis with keratinocyte (...) necrosis and intraepidermal acantholysis. Direct immunofluorescence exhibited deposition of intercellular IgG and complement and granular complement at the dermoepidermal junction. Indirect immunofluorescence testing showed a typical intercellular staining on monkey esophagus and rat bladder epithelium. Immunoprecipitation showed characteristic target antigens of 250, 210, and 190 kDa molecular weights. This patient met all diagnostic criteria for paraneoplastic pemphigus and is, to our knowledge

2003 Journal of American Academy of Dermatology

207. Paraneoplastic pemphigus in association with Castleman's disease. (Abstract)

Paraneoplastic pemphigus in association with Castleman's disease. Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with lymphoproliferative neoplasms, and frequently with a very rare tumour, Castleman's disease.To analyse the clinical history, immunopathological and histopathological findings in 28 patients with a confirmed diagnosis of PNP and Castleman's disease.Sera from all patients were assayed by indirect immunofluorescence (IF) and immunoprecipitation (IP

2003 British Journal of Dermatology

208. Paraneoplastic pemphigus in children and adolescents. (Abstract)

Paraneoplastic pemphigus in children and adolescents. Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with specific B-cell lymphoproliferative neoplasms. There has been an increasing number of individual reports in the childhood and adolescent population.To examine the clinical and immunopathological features of PNP occurring in children and adolescents.We analysed the clinical and immunopathological findings of 14 patients under the age of 18 years

2002 British Journal of Dermatology

209. Paraneoplastic pemphigus associated with uterine carcinoma. (Abstract)

Paraneoplastic pemphigus associated with uterine carcinoma. Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease characterized by severe mucous membrane involvement, polymorphous skin eruptions, and underlying neoplasms, usually those of lymphoreticular system. Cases of PNP associated with solid cancer are extremely rare. A 46-year-old woman presented with severe stomatitis, pseudomembranous conjunctivitis, and polymorphous skin eruptions. Histologic, immunofluorescence

2003 Journal of American Academy of Dermatology

210. Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. (Abstract)

Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Pemphigus is a bullous mucocutaneous autoimmune disease characterized by IgG autoantibodies to desmoglein (Dsg) 1 and/or Dsg3. Occasionally direct immunofluorescence of pemphigus skin reveals IgA depositions with an intraepidermal intercellular pattern in addition to the IgG deposition.To investigate if pemphigus patients, in addition to having IgG autoantibodies, also (...) ) and patients who had paraneoplastic pemphigus (PNP) (n=8).IgA antibodies to Dsg1 were found in 13 (38%) of the patients with IgG to Dsg1, in five (16%) of the patients with IgG to both Dsg1 and Dsg3, in four (15%) of the patients with IgG to Dsg3 and in none of the PNP patients. IgA antibodies to Dsg3 were found in one (3%) of the patients with IgG to Dsg1, in 18 (58%) of the patients with IgG to both Dsg1 and 3, in 18 (67%) of the patients with IgG to Dsg3, and in four (50%) of the PNP patients

2007 British Journal of Dermatology

211. Paraneoplastic pemphigus associated with Castleman tumor: a commonly reported subtype of paraneoplastic pemphigus in China. Full Text available with Trip Pro

Paraneoplastic pemphigus associated with Castleman tumor: a commonly reported subtype of paraneoplastic pemphigus in China. Castleman tumor, a rare lymphoproliferative disorder, is one of the associated tumors in paraneoplastic pemphigus. We analyzed the characteristics of a group of patients with Castleman tumor to clearly understand and to improve the prognosis of the disease.Ten cases of paraneoplastic pemphigus associated with Castleman tumor treated in the Department of Dermatology, Peking (...) University First Hospital, Beijing, China, from May 1, 1999, to March 31, 2004, were analyzed for clinical aspects, characteristics and histologic features of the tumors, and computed tomographic findings. Literature was reviewed and data were compared with our cases. Castleman tumor was a frequently reported neoplasm in association with paraneoplastic pemphigus in China. The disease was found to be caused by an autoimmune reaction originating from the B lymphocytes in the Castleman tumor.Castleman tumor

2005 Archives of Dermatology

212. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. Full Text available with Trip Pro

Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that occurs in association with underlying neoplasms. Patients with PNP develop characteristic IgG autoantibodies directed against multiple antigens, most of which have been identified as cytoplasmic proteins of the plakin family (desmoplakin I, II, BPAG1 (...) , envoplakin, and periplakin). This study identified cell surface target antigens of PNP. We focused on desmoglein (Dsg) 3 and Dsg1, the autoantigens of pemphigus vulgaris and pemphigus foliaceus. ELISA using baculovirus-expressed recombinant Dsgs (rDsg3, rDsg1) has revealed that 25 out of 25 PNP sera tested were positive against Dsg3 and 16 of 25 were positive against Dsg1. All of 12 PNP sera tested immunoprecipitated Dsg3. Removal of anti-Dsg3 autoantibodies by immunoadsorption was sufficient

1998 Journal of Clinical Investigation

213. A nonfatal case and 2 fatal cases of paraneoplastic pemphigus: can a complement indirect immunofluorescent test help to identify fatal "group A" paraneoplastic pemphigus cases? (Abstract)

A nonfatal case and 2 fatal cases of paraneoplastic pemphigus: can a complement indirect immunofluorescent test help to identify fatal "group A" paraneoplastic pemphigus cases? We studied 3 recent cases of paraneoplastic pemphigus (PNP) in detail. Two patients died despite concerted management efforts. One patient received no treatment after the appearance of PNP and recovered completely from both PNP and lymphoma. Multiple serum studies of these 3 patients plus 9 other proven PNP cases (...) revealed that 8 of 9 fatal PNP cases (referred to here as "group A") had distinctive cell surface antibodies detected by complement indirect immunofluorescent (CIIF) tests on monkey esophagus sections. By contrast, none of the sera from 3 patients with PNP who experienced long-term survival (referred to here as "group B") and none of 20 pemphigus vulgaris or 10 pemphigus foliaceus control sera revealed similar beaded cell surface CIIF reaction patterns, a difference that is statistically significant (P

2002 Journal of American Academy of Dermatology

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