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Pemphigus Foliaceus

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321. Three cases of transition from pemphigus vulgaris to pemphigus foliaceus confirmed by desmoglein ELISA. (PubMed)

Three cases of transition from pemphigus vulgaris to pemphigus foliaceus confirmed by desmoglein ELISA. We report 3 cases of pemphigus vulgaris (PV) confirmed by histology and direct and indirect immunofluorescence that showed transition to pemphigus foliaceus (PF) 2-4 years from the time of disease onset. Desmoglein (Dsg) ELISA testing of the sera from these 3 patients in the later stages of their disease showed the presence of anti-Dsg1 antibodies and the absence of anti-Dsg3 antibodies

2005 Dermatology

322. Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil. (PubMed)

Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil. Mycophenolate mofetil is increasingly being used as a corticosteroid-sparing agent in immunosuppressive regimens.To elucidate the effectiveness of mycophenolate as adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus.Historical prospective study.University hospital.The study included 42 consecutive patients with pemphigus (31 with pemphigus vulgaris and 11 with pemphigus foliaceus (...) ) who had relapses during prednisone taper or had clinically significant adverse effects from previous drug therapy.Remission was achieved in 22 (71%) and 5 (45%) of patients with pemphigus vulgaris and pemphigus foliaceus, respectively. Partial remission was achieved in 1 (3%) and 4 (36%), respectively. The median time to achieve complete remission was 9 months (range, 1-13 months). The treatment was administered for a median of 22 months, and the median follow-up period was 22 months. Seventy

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2003 Archives of Dermatology

323. The IgM Anti-Desmoglein 1 Response Distinguishes Brazilian Pemphigus Foliaceus (Fogo Selvagem) from Other Forms of Pemphigus. (PubMed)

The IgM Anti-Desmoglein 1 Response Distinguishes Brazilian Pemphigus Foliaceus (Fogo Selvagem) from Other Forms of Pemphigus. Fogo selvagem (FS) and pemphigus foliaceus (PF) possess pathogenic IgG anti-desmoglein 1-(Dsg1) autoantibodies. Although PF occurs sporadically, FS is endemic in Limao Verde (LV), Brazil (3.4% prevalence). IgM anti-Dsg1 were detected in 58% FS LV patients (n=31), 19% of FS patients from Hospital-Campo Grande (n=57), 19% from Hospital-Goiania (n=42), 12% from Hospital-Sao (...) Paulo (n=56), 10% of PF patients from United States (n=20), and 0% of PF patients from Japan (n=20). Pemphigus vulgaris (n=40, USA and Japan), bullous pemphigoid (n=40, USA), and healthy donors (n=55, USA) showed negligible percentages of positive sera. High percentages of positive IgM anti-Dsg1 were found in healthy donors from four rural Amerindian populations (42% of 243) as compared with urban donors (14% of 81; P<0.001). More than 50% of healthy donors from LV (n=99, age 5-20 years) possess IgM

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2007 Journal of Investigative Dermatology

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

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 (...) generate IgA antibodies to Dsg1 and/or Dsg3.Sera of 100 pemphigus patients and 36 bullous pemphigoid controls were tested by IgA enzyme-linked immunosorbent assay (ELISA) to the recombinant extracellular domains of Dsg1 and Dsg3. The patients were selected on clinical grounds and positive IgG ELISA index values for Dsg1 and/or Dsg3. They were divided into four groups: patients having IgG to only Dsg1 (n=34), patients having IgG to both Dsg1 and Dsg3 (n=31), patients having IgG to only Dsg3 (n=27

2007 British Journal of Dermatology

325. An interesting case of pemphigus foliaceus in a dog. (PubMed)

An interesting case of pemphigus foliaceus in a dog. 10065323 1999 03 23 2018 11 13 0008-5286 40 2 1999 Feb The Canadian veterinary journal = La revue veterinaire canadienne Can. Vet. J. An interesting case of pemphigus foliaceus in a dog. 127-8 High M M Sydney Veterinary Services, British Columbia. eng Case Reports Journal Article Canada Can Vet J 0004653 0008-5286 0 Antirheumatic Agents 2P2V9Q0E78 Aurothioglucose IM Animals Antirheumatic Agents therapeutic use Aurothioglucose therapeutic use (...) Dog Diseases drug therapy Dogs Female Pemphigus drug therapy veterinary 1999 3 5 1999 3 5 0 1 1999 3 5 0 0 ppublish 10065323 PMC1539552 J Am Vet Med Assoc. 1985 Jan 1;186(1):59-66 3880732 J Am Vet Med Assoc. 1985 Jan 1;186(1):67-9 3965427

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1999 The Canadian Veterinary Journal

326. Pemphigus foliaceus (PubMed)

Pemphigus foliaceus Pemphigus foliaceus is a skin disease in which antibodies against the cell surface of keratinocytes destroy the adhesion between epidermal cells, thereby producing blisters. It is a rare disease in childhood, and treatment guidelines for juvenile pemphigus foliaceus are lacking. An 8 year old boy with pemphigus foliaceus is described. He did not respond to topical steroids, and the condition flared up when high dose oral steroids were tapered. The lesions resolved completely

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1997 Archives of Disease in Childhood

327. Desmoglein-1–specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem) (PubMed)

Desmoglein-1–specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem) Fogo selvagem (FS), the endemic form of pemphigus foliaceus, is a cutaneous autoimmune disease characterized by subcorneal blistering of the epidermis and the production of autoantibodies against the desmosomal antigen desmoglein-1 (Dsg1). Previously, we showed that mice injected with autoantibodies from FS patients develop a skin disease that reproduces the clinical, histological

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2000 Journal of Clinical Investigation

328. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris (PubMed)

Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris Patients with pemphigus foliaceus (PF) have blisters on skin, but not mucous membranes, whereas patients with pemphigus vulgaris (PV) develop blisters on mucous membranes and/or skin. PF and PV blisters are due to loss of keratinocyte cell-cell adhesion in the superficial and deep epidermis, respectively. PF autoantibodies are directed against desmoglein (Dsg) 1; PV autoantibodies bind Dsg3 (...) or both Dsg3 and Dsg1. In this study, we test the hypothesis that coexpression of Dsg1 and Dsg3 in keratinocytes protects against pathology due to antibody-induced dysfunction of either one alone. Using passive transfer of pemphigus IgG to normal and DSG3(null) neonatal mice, we show that in the areas of epidermis and mucous membrane that coexpress Dsg1 and Dsg3, antibodies against either desmoglein alone do not cause spontaneous blisters, but antibodies against both do. In areas (such as superficial

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1999 Journal of Clinical Investigation

329. Monovalent Fab' immunoglobulin fragments from endemic pemphigus foliaceus autoantibodies reproduce the human disease in neonatal Balb/c mice. (PubMed)

Monovalent Fab' immunoglobulin fragments from endemic pemphigus foliaceus autoantibodies reproduce the human disease in neonatal Balb/c mice. Fogo selvagem (FS) is an autoimmune disease caused by IgG autoantibodies to desmoglein I (DG-I), a desmosomal glycoprotein. We have previously shown that the autoantibodies in these patients are pathogenic and restricted mainly to the IgG4 subclass. The purpose of this study was to determine if the Fc domain or the valence of FS autoantibodies were

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1990 Journal of Clinical Investigation

330. Distinction between epidermal antigens binding pemphigus vulgaris and pemphigus foliaceus autoantibodies. (PubMed)

Distinction between epidermal antigens binding pemphigus vulgaris and pemphigus foliaceus autoantibodies. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases in which antibodies develop to the cell surface of epidermal cells. In this study we sought to determine the antigenic specificity of antibodies in the sera of patients with PV and PF. Sera from 12 patients with PV were used to immunoprecipitate extracts of cultured human epidermal cells that were

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1984 Journal of Clinical Investigation

331. Identification of pemphigus vulgaris antigen extracted from normal human epidermis and comparison with pemphigus foliaceus antigen. (PubMed)

Identification of pemphigus vulgaris antigen extracted from normal human epidermis and comparison with pemphigus foliaceus antigen. Immunoprecipitations of cultured keratinocyte extracts have shown that pemphigus vulgaris (PV) sera bind a polypeptide of 210,000 mol wt with disulfide-linked chains of 130,000 and 85,000 mol wt. To identify proteins in normal human skin recognized by PV antibodies, we performed immunoprecipitations of normal human epidermal extracts. All 22 PV sera tested (...) to be precipitated by all pemphigus foliaceus (PF) sera. The PF complex consists of polypeptides of 260,000, 160,000, 110,000, and 85,000 mol wt. Although the majority of PV sera also precipitate the PF complex, no PF sera precipitate the PV complex. Thus, PV and PF can be absolutely distinguished on a molecular level using the patients' autoantibodies. The PV and PF complexes, although distinct, have certain similarities. The 85,000-mol-wt polypeptide of each is identical. The 160,000-mol wt-peptide of the PF

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1988 Journal of Clinical Investigation

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