PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “PEMPHIGUS FOLIACEUS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 145 records · Page 8Linked to original sources
PubMed2003

Comparative study of autoantigen profile between Colombian and Brazilian types of endemic pemphigus foliaceus by various biochemical and molecular biological techniques.

BACKGROUND: Besides Brazilian endemic pemphigus foliaceus (EPF), we have described another focus of EPF in Colombia. Our previous study suggested that Colombian EPF seemed to react various plakin family proteins, such as envoplakin, periplakin and BP230. OBJECTIVE: To further characterize the Colombian EPF and study the difference from Brazilian EPF, we examined the antigen profile of the two types of EPF. METHODS AND RESULTS: Immunoblotting using normal human epidermal extracts revealed that 38% Colombian EPF sera and 25% Brazilian EPF sera showed IgG antibodies reactive with desmoglein (Dsg) 1, pemphigus foliaceus antigen. The sera of both types of EPF showed protein bands co-migrating with plakin family proteins, particularly periplakin. Immunoblotting analyses using recombinant proteins of various domains of envoplakin, periplakin and BP230 revealed that a considerable number of Colombian EPF sera reacted with recombinant proteins of periplakin, while only few Brazilian sera reacted with some of the recombinant proteins of any plakins. Enzyme-linked immunosorbent assay (ELISA) for Dsg1 and Dsg3 showed that Dsg1 was reacted by almost all sera of both types of EPF. However, unexpectedly, while none of Colombian EPF sera reacted with Dsg3, about half of Brazilian EPF sera reacted with Dsg3. CONCLUSION: These results suggested that the Colombian EPF is basically similar to Brazilian EPF in terms that major antigen is Dsg1, but there were some different antigen profiles between the two types of EPF.

Autoantibodies↗
PubMed1989

Are HLA class II genes controlling susceptibility and resistance to Brazilian pemphigus foliaceus (fogo selvagem)?

In order to investigate the possible association between Brazilian pemphigus foliaceus and HLA, we studied 48 patients and 74 matched controls, all Brazilian Caucasoids, for HLA-A,B,C; DR1 to DRw8 and DQw1 to DQw3. The frequencies of DR1, DR4 and B16 were significantly increased, while DR7 was significantly decreased among the patients. Furthermore DQw2, likewise the DR specificities associated with it - DR3 and DR7 - never occurred among the patients in the absence of the susceptibility markers DR1, DQw1 or DR4, DQw3. Acting on these findings, we suggest that at least two MHC-class II genes are involved in the pathogenesis of Brazilian pemphigus foliaceus: at least one gene, associated to DR1,DQw1 and to DR4,DQw3, confers susceptibility and at least one gene, associated to DR7,DQw2 and DR3,DQw2, confers resistance. The susceptibility gene(s) seem(s) to be epistatic to or dominant over (if allelic) the resistance gene(s). Both are dominant over other alleles at their locus (or loci).

Adolescent↗
PubMed2003

Polymorphisms within the tumor necrosis factor and lymphotoxin-alpha genes and endemic pemphigus foliaceus--are there any associations?

The purpose of this study was to analyze the possible influence of the TNF and LTA loci polymorphisms on the susceptibility/resistance to endemic pemphigus foliaceus, also named fogo selvagem (FS), an autoimmune disease characterized by blisters due to acantholysis of the superficial-most epidermal cells. Autoantibodies, mainly of the IgG4 subclass, are directed against a desmosomal glycoprotein known as desmoglein 1. FS shares clinical, histological and immunological features with nonendemic pemphigus foliaceus. Most residents of the endemic regions do not develop the disease, and familial clustering has been documented, suggesting that host factors play a role in susceptibility. In fact, strong positive and negative associations with HLA class II genes have been reported. The TNF and LTA genes are located in the class III region of the Human Major Histocompatibility Complex. Their location, the function of their products, which are cytokines and pluripotent immunomodulators, as well as their genetic variability make them candidate genes for complex diseases with an altered immune response. A total of 162 patients and 191 controls were enrolled in this study. No significant associations were found with any one of the three LTA single nucleotide polymorphisms (SNP) analyzed (at nucleotides 249, 365, 720), nor with the TNF SNP located at positions -863 and -308. The frequency of allele TNF*238A was slightly decreased in patients (OR = 0.45). In conclusion, the results of this study indicate that genetic variability of the TNF and LTA genes does not play a major role in susceptibility/resistance to pemphigus foliaceus.

Alleles↗
PubMed1996

Pemphigoid and pemphigus foliaceus successfully treated with topical corticosteroids.

Six patients with pemphigoid and three patients with pemphigus foliaceus were successfully treated with topical corticosteroids. This was especially effective in cases of pretibial localized pemphigoid and in mild cases of bullous pemphigoid and pemphigus foliaceus with negative or low-titer circulating autoantibodies.

Administration, Topical↗
PubMed1985

Coexistence of pemphigus foliaceus and mycosis fungoides.

Mycosis fungoides was documented in a patient two years after pemphigus foliaceus had been diagnosed and treated with corticosteroids. Eight years later, the patient was found to have stage IV lymphomatous disease with generalized erythroderma and palpable, histologically positive lymph nodes. Hematoxylin-eosin staining of a specimen of erythroderma revealed mycosis fungoides, while direct immunofluorescence of this tissue revealed intercellular IgG deposits diagnostic of pemphigus foliaceus.

Aged↗
PubMed1977

Pemphigus foliaceus associated with absence of intercellular antigens in lower layers of epidermis.

A patient with pemphigus foliaceus was found to lack normal intercellular (IC) antigens in the lower layers of the epidermis. This was evidenced by the inability of her own IC antibodies, or of those from other patients with pemphigus vulgaris, to bind to the IC substance in the lower layers of her epidermis; whereas these same antibodies reacted to IC antigens in all layers of normal allogeneic skin and monkey and guinea pig esophagus. Lack of IC antigens in the lower layers of the epidermis may account for the subcorneal location of bullae in some patients with pemphigus foliaceus.

Antigens↗
PubMed1981

Penicillamine-induced pemphigus foliaceus. A fatal outcome.

Penicillamine-induced pemphigus is an uncommon but well-recognized phenomenon. It has been generally believed to be a benign disease responding to withdrawal of the drug or to treatment with a short course of oral steroids. In the hope that it will increase awareness of the potential severity of this disease, we report herein a case of penicillamine-induced pemphigus foliaceus with fatal termination.

Female↗
PubMed2004

Outcome and complications associated with treatment of pemphigus foliaceus in dogs: 43 cases (1994-2000).

OBJECTIVE: To identify factors affecting prognosis, outcome, and complications associated with pemphigus foliaceus in dogs. DESIGN: Retrospective study. ANIMALS: 43 dogs with pemphigus foliaceus. PROCEDURE: Medical records were reviewed for signalment, age at diagnosis, duration to diagnosis, body area affected, initial immunosuppressive regimens and concurrent use of antimicrobials and sucralfate or histamine receptor 2 blocking agent, adverse effects of treatment, duration of treatment, number of visits for follow-up care, cause of death, and credentials of the veterinarians responsible for continued care. RESULTS: The case fatality rate was 60.5%. Factors significantly correlated with survival time included concurrent use of antimicrobials during initiation of immunosuppressive treatment and a lower number of adverse effects to treatment. Treatment times lasting more than 10 months from diagnosis correlated significantly with survival. CONCLUSIONS AND CLINICAL RELEVANCE: Treatment with or prophylactic use of antimicrobials may be warranted during initial immunosuppressive treatment. The inverse correlation between survival time and number of adverse treatment effects was not unexpected because it was reflective of the owners' decision to euthanatize their dogs and of corticosteroid-related secondary diseases. Survival beyond the tenth month of treatment predicted long-term survival, which suggests that dogs require careful management during the early months of treatment.

Animals↗
PubMed1976

Pemphigus foliaceus. Early clinical appearance as dermatitis herpetiformis with eosinophilic spongiosis.

We encountered an unusual case of pemphigus foliaceus in which there were early clinical signs of dermatitis herpetiformis with eosinophilic spongiosis histologically. Numerous authors have appreciated that eosinophilic spongiosis occurs in early pemphigus foliaceus, usually in the preacantholytic stage when the lesions may clinically simulate dermatitis herpetiformis. Recognition of eosinophilic spongiosis may help differentiate early pemphigus from other vesiculobullous disorders.

Acantholysis↗
PubMed1997

Pemphigus foliaceus: preferential binding of IgG1 and C3 at the upper epidermis.

The immunopathological mechanism of pemphigus foliaceus acantholysis remains controversial. Previous workers have found that IgG autoantibodies are reactive with the epidermal surface and capable of blister induction. The presence of complement components in lesions also suggests their potential role in this disease. Complement participation, however, has not been clearly demonstrated. We describe a case of pemphigus foliaceus (PF) in a 58-year old woman which was confirmed by histopathological and immunopathological studies, and by ELISA assay on recombinant PF antigen. Unusual features of this case include the predominance of IgG1 anti-epithelial antibody in both the deposits and circulation, and the preferential binding of IgG and complement component 3 at the upper epidermis. We believe that this pattern of co-localized deposition suggests a location-specific, non-random complement activation.

Autoantibodies↗
PubMed2002

The transition of pemphigus vulgaris into pemphigus foliaceus: a reflection of changing desmoglein 1 and 3 autoantibody levels in pemphigus vulgaris.

The transition of pemphigus vulgaris (PV) into pemphigus foliaceus (PF) is rare and the immunological changes underlying this event are not well understood. We report a 44-year-old woman who presented with oral and cutaneous erosions typical of PV. Over a 9-year period, the clinical features evolved into those of PF. To examine whether quantitative changes in desmoglein (Dsg) antibodies were associated with this transition, Dsg1 and Dsg3 antibody levels were measured by enzyme-linked immunosorbent assay in 82 sequential serum samples collected over this period. At presentation, when the phenotype was PV with oral and cutaneous erosions, antibodies to both Dsg1 and Dsg3 were detected. The disappearance of oral involvement was associated with a decline in Dsg3 antibodies, which are now undetectable, while the development of more severe skin involvement was associated with rising Dsg1 antibody levels. These data strongly suggest that the change in clinical features is a reflection of qualitative and quantitative changes in antibody profile. It is not known whether the transition to PF is permanent or whether disease relapses in the future may be associated with the re-emergence of Dsg3 antibodies, oral ulceration and a PV phenotype.

Adult↗
PubMed2002

T cell receptor Beta chain gene usage in endemic pemphigus foliaceus (fogo selvagem).

The trimolecular complex comprised of the major histocompatibility complex, peptide antigen, and the T cell receptor is a requisite for T cell activation in normal and autoimmune responses. T cell receptor analysis is critical to further our understanding regarding mechanisms of T cell epitope selection and autoimmune initiation and progression and may help to identify targets for immunotherapy. Pemphigus foliaceus is an autoimmune blistering skin disease characterized by intraepidermal blisters and circulating autoantibodies directed against desmoglein 1, a 160 kDa transmembrane desmosomal molecule expressed in keratinocytes. As tissue damage is mediated by anti-desmoglein 1 antibodies, an initial T cell response is a likely requirement for autoantibody generation in this disease. To elucidate the role of pathogenic T cells in autoimmunity further, we have directly characterized the T cell receptor of T cells derived from pemphigus foliaceus patients. Complementary DNA was isolated from 17 desmoglein 1 specific T cell clones generated from pemphigus foliaceus patients by clonal expansion in vitro. To analyze the T cell repertoire, a panel of primers, collectively specific for the known human T cell receptor beta variable region (TCRBV) families were paired with a constant region primer to polymerase chain reaction to amplify one distinct T cell receptor beta variable region allele for each T cell clone studied. Polymerase chain reaction products were sequenced to determine exact beta chain gene usage. In the 17 clones tested, 10 distinct T cell receptor beta variable region usages and nine T cell receptor beta joining gene segment usages were identified. Furthermore, T cell receptor beta variable region and beta joining usage did not appear to be random, but oligoclonal in nature, with some preference shown for T cell receptor beta variable region 5S1 and T cell receptor BJ2S5.

Amino Acid Sequence↗
PubMed2006

Pemphigus foliaceus: a case report and short review.

Pemphigus foliaceus (PF) is a rare autoimmune blistering disease presenting in endemic and sporadic forms. The typical presentation is recurrent shallow erosions in a seborrheic distribution. We present a case of a 58-year-old woman with PF who was successfully treated with a combination of oral corticosteroids and dapsone. We also provide a concise review of the literature and discuss the etiology, clinical features, diagnosis, and management of PF.

Anti-Inflammatory Agents↗
PubMed2005

Pemphigus foliaceus masquerading as postoperative wound infection: report of a case and review of the Koebner and related phenomenon following surgical procedures.

BACKGROUND: The Koebner phenomenon, also known as the isomorphic response, is the development of preexisting skin disease following trauma to uninvolved skin. Various cutaneous disorders have been described to arise at surgical wounds and scars. Moreover, dermatologic procedures, such as cold-steel and laser surgery, can evoke koebnerization. OBJECTIVE: To describe a case of pemphigus foliaceus arising in postoperative wounds and to present a review of dermatologic disorders triggered by surgical procedures. METHODS: We report a case of pemphigus foliaceus initially presenting at sites of Mohs' micrographic surgery, shave biopsy, and cryotherapy and, subsequently, at a nonsurgical site. We reviewed the English literature in MEDLINE from November 1955 to April 2004 for reports of Koebner and related phenomenon following surgical procedures. RESULTS: To our knowledge, this is the first reported case of pemphigus foliaceus erupting at surgical and cryotherapy wounds. The clinical appearance can mimic wound infection. In addition to inducing preexisting disease, cutaneous procedures can also trigger the onset of new disease, which can either be limited only to the surgical site or subsequently become generalized. CONCLUSION: Postoperative Koebner or related responses should be included in the differential diagnosis of poorly healing surgical wounds. Skin biopsies for histopathology and immunologic studies may be necessary for definitive diagnosis and optimal management.

Aged↗
PubMed1995

Antigen-specific immunoadsorption of pathogenic autoantibodies in pemphigus foliaceus.

Patients with the autoimmune blistering disease pemphigus foliaceus (PF) have circulating autoantibodies directed against the desmosomal cadherin desmoglein 1 (Dsg1). Based on the fact that purified IgG fractions from PF patients induce loss of cell adhesion in organ culture and in a neonatal mouse model, it has been proposed that these anti-Dsg1 antibodies play a pathogenic role in blister formation. To directly address whether antibodies in PF sera specific for the Dsg1 extracellular domain are indeed pathogenic in the disease, PFIg, a chimeric protein containing the entire extracellular domain of human Dsg1 and the constant region of human IgG1, was produced by baculovirus expression. Incubation of PF patients' sera with the PFIg baculoprotein removed the immunoreactivity of autoantibodies against keratinocyte cell surfaces in all 20 PF and eight Brazilian PF patients' sera tested. This adsorption was conformation dependent, because PFIg protein denatured by low pH or heat was no longer able to adsorb the immunoreactivity of PF sera. Furthermore, the incubation with the PFIg baculoprotein eliminated the pathogenic activity of PF patients' sera and prevented gross blister formation in a neonatal mouse model of pemphigus. Anti-Dsg1 antibodies eluted from the PFIg protein column were pathogenic as they resulted in the appearance of gross blisters in neonatal mice with typical histologic findings of PF. These observations indicate that the extracellular domain of Dsg1 expressed by baculovirus is capable of specifically immunoadsorbing pathogenic autoantibodies from PF patients' sera and provide direct evidence that the anti-Dsg1 autoantibodies in PF sera are indeed pathogenic. The availability of this Dsg1 recombinant protein may facilitate the development of antigen-specific plasmapheresis as a novel therapeutic strategy in pemphigus.

Animals↗
PubMed1976

T and B lymphocytes in South American pemphigus foliaceus.

T and B lymphocytes were quantified in peripheral blood of thirty patients with South American pemphigus foliaceus according to their ability to form rosettes with sheep erythrocytes (E) or sheep erythrocytes sensitized with antibody an complement (EAC). When compared with the counts obtained from thirty normal subjects, a decrease was found in the total T-lymphocyte count (32-33+/-7-7 versus 46-7+/-8-7) and in the T functional lymphocyte count as detected by the active rosette test. The mean percentage of B lymphocytes within the total number of lymphocytes was not significantly different from that of normal subjects (24-0 +/- 8-2 versus 25-3+/-8-0). Lymph node sections from three pemphigus patients examined for E or EAC adherence showed depletion of T cells in the paracortical areas. The low percentage of E cells in the peripheral blood and the depletion of E cells in paracortical areas of lymph nodes from patients with South American pemphigus foliaceus may reflect an impaired cellular immunity.

Adult↗
PubMed2005

Desmoglein-1 is a minor autoantigen in dogs with pemphigus foliaceus.

The majority of human patients with pemphigus foliaceus (PF) have circulating IgG autoantibodies that target conformational epitopes on the desmosomal cadherin desmoglein-1 (dsg1). Limited studies using immunoblot techniques suggested that the principal autoantigen in dogs with PF might also be dsg1. It was the objective of this study to test this hypothesis. A comprehensive survey of canine PF sera was conducted using a novel screening strategy that detects conformational epitopes. This method consists of the ectopic expression of canine dsg1 at the surface of human 293T epithelial kidney cells and their live screening, i.e. prior to fixation. Out of seven control human PF sera that bound to canine epidermis, three (57%) contained IgG autoantibodies that recognized ectopically expressed canine dsg1 with a membrane and punctate pattern. Out of 83 canine PF sera only five (6%) contained IgG that recognized canine dsg1. Consistent with findings for human PF sera obtained in this study, autoantibody binding was conformation- and glycosylation-dependent as demonstrated by calcium chelation with EDTA and tunicamycin or wheat germ agglutinin treatment, respectively. In conclusion, these studies establish canine dsg1 as a minor autoantigen for canine PF. Antigenic epitopes appear to be conformation- and glycosylation-dependent.

Animals↗