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 235 records · Page 13Linked to original sources
PubMed1994

A non-desmoglein component of bovine epidermis reactive with pemphigus foliaceus sera.

A component from bovine epidermis has been purified and found to react with sera from patients with pemphigus foliaceus (PF). This component appears to have molecular weight characteristics similar to those of a previously reported component of human and bovine epidermis which was reactive with PF sera, but only detectable by radiolabeling. It is composed of an 80 kD and a 45 kD polypeptide which are thought to be covalently linked. By chromatographic data, the native form appears to be a noncovalently joined dimer of the 80 and 45 kD polypeptides. Amino acid analysis and sequence data indicate that the 80 kD component is not desmoglein (DG) nor any other known protein whose sequence is entered in data banks. Previous studies by Stanley indicate that DG is the major target of autoantibodies in PF sera. Our finding of another epidermal component reactive with PF antibodies raises some questions. It is not yet known if PF anti-DG antibodies cross-react with this new epidermal antigen or if these antibodies constitute separate and non-cross-reacting sets. Both possibilities and their ramifications are discussed.

Amino Acid Sequence↗
PubMed1987

Distinguishing the antigen specificities of pemphigus vulgaris and pemphigus foliaceus using the biotin-avidin immunofluorescence method.

To compare the specificities of autoantibodies in sera from patients with pemphigus vulgaris (PV) and those with pemphigus foliaceus (PF), blocking-immunofluorescence studies were carried out using the biotin-avidin immunofluorescence technique. First cryostat sections of bovine muzzle epidermis were incubated with one of either the unlabeled PV or PF serum samples for 2 h at room temperature, then rinsed and overlayered for 30 min with serially diluted corresponding or other biotin-labeled PV (or PF) IgG fractions containing their autoantibodies. The sections were then incubated for 30 min in fluorescein-labeled avidin. The blocking abilities of PV (or PF) sera for the reaction of labeled PV (or PF) IgG on the membranous part of keratinocytes were compared. The following results were obtained: the titers of biotin-labeled PF IgG decreased considerably more in sections preincubated with PF sera than sections preincubated with PV or normal sera. The titers of biotin-labeled PV IgG decreased considerably more in sections preincubated with PV sera than sections preincubated with PF or normal sera. These results suggest that there may be a distinction in antigenic specificities between PV and PF sera.

Autoantibodies↗
PubMedDate not supplied

[Indirect immunofluorescence in endemic pemphigus foliaceus. A contribution to its standardization].

The aim of the present research was to standardize the indirect immunofluorescence reaction for Endemic Pemphigus Foliaceus (Fogo Selvagem). We found that fresh human skin was the ideal substrate and could proceed from foreskin, head, neck, or anterior abdominal wall. PBS pre-washing of the skin preceding the incubation with the serum should be avoided since the antigenicity might be diminished. TAS-calcium pre-serves the Pemphigus antigenic properties of the skin and shall be preferred as the diluent for the sera. Albumin-coated slides are useful because they increase the adherence of the skin sections. The conjugate appropriate dilution is convenientely determined by the radial immunodiffusion test (Ouchterlony method). So far as the correlation between the antibody titer and the clinical activity is concerned, we concluded that a titer of 160 or more was of bad prognosis, since it was associated with the generalized form of the disease or with cases of the localized form refractory to the usual therapy. Nevertheless, this assumption needs confirmation by further studies involving an appropriate clinical approach.

Autoantibodies↗
PubMedDate not supplied

Endemic Pemphigus foliaceus ("Fogo selvagem"): a series from the Northeastern region of the State of São Paulo, Brazil, 1973-1998.

UNLABELLED: Endemic Pemphigus Foliaceus (EPF) is a bullous autoimmune skin disease whose incidence used to be high in the State of São Paulo (SP), Brazil, during the forties, but has declined thereafter. OBJECTIVES: to report a series of EPF patients from the northeastern region of SP. METHODS: a retrospective study concerning demographic and epidemiological data of patients seen from 1973 to 1998 was conducted at the University Hospital, Faculty of Medicine of Ribeirão Preto, SP. RESULTS: bullous disease was diagnosed in 340 patients, 245 with EPF (72.1%), 9.4 cases per year, 60.4% females, and 70.2% white, 7 to 82 year-old (29.4% in their teens); 46.9% lived in the rural zone. Concerning profession, housewives predominated among women (67.6%) and agricultural workers among men (40.2%). The time of disease was less than 1 year in 62.0% of cases, followed by 1 and 5 years (27%), and more than 5 years for the remaining patients (11%). 36.7% of patients were referred by the Direção Regional de Saúde (DIR) XVIII of Ribeirão Preto, with the largest number of cases being from Ribeirão Preto and Batatais: 33.3% and 23.3%, respectively; 22% from DIR XIII (Franca); 13.5% from DIR VII (Araraquara); 2.9% from DIR IX (Barretos); 4.1% from other DIRs of SP, and 20.8% from other States (16.7% from Minas Gerais). Thirteen (5.3%) patients reported occurrence of the disease in some relative, and 4 (1.6%) in neighbors. CONCLUSIONS: the present data characterize the northeastern region of the state of São Paulo as a remaining endemic focus of EPF.

Adolescent↗
PubMed1989

Endemic pemphigus foliaceus (Fogo Selvagem): II. Current and historic epidemiologic studies.

This paper details current and historic epidemiologic features of Fogo Selvagem (Endemic pemphigus foliaceus) in Brazil. The following features are described. a) The disease occurs in endemic fashion in regions of Brazil within the states of Goias, Mato Grosso do Sul, Parana, Sao Paulo, and Minas Gerais. It appears that the disease is spreading toward the northwest and west, involving the states of Mato Grosso, Para, Maranhao, Rondonia, Acre, and Amazonas. b) People at risk are young peasants or children of either sex or any race exposed to the local ecology in rural areas of endemic states. Although the disease has been described in urban centers, these occurrences are rare. c) Fogo Selvagem commonly appears in wild areas being colonized and disappears as these areas become urbanized. d) The majority of patients live in close proximity to rivers and within the 10-15 Km flying range of mosquitos or black flies (such as Simulium). It is hypothesized that a black fly, Simulium pruinosum may be the vector that precipitates the disease. f) There is a significant number of Fogo Selvagem in family units where multiple, genetically related individuals are affected. g) Finally, autoantibodies against lupus-associated antigens are not present in the sera of patients with Fogo Selvagem. Clinical examination of the skin, and serologic screening for pemphigus autoantibodies are specific parameters that can be used in the search for the etiologic agents that lead to autoimmune disease of the skin. To identify and prove an etiologic agent for this well-characterized autoimmune disease would be of tremendous importance to the understanding of autoimmune skin diseases, and potentially other organ-specific autoimmune disorders.

Age Factors↗
PubMed2006

Changes in the autoimmune blistering response: a clinical and immunopathological shift from pemphigus foliaceus to bullous pemphigoid.

We describe a 64-year-old Brazilian man who developed bullous pemphigoid (BP) 12 years after pemphigus foliaceus (PF) was diagnosed. On his first presentation in 1992, histological examination revealed intraepidermal blistering and acantholysis at the granular layer, direct immunofluorescence (DIF) demonstrated intercellular deposits of C3 in the epidermis, and indirect immunofluorescence showed the presence of IgG antibodies against the intercellular spaces. In 2004, laboratory findings revealed a subepidermal blister with neutrophils and eosinophils (by histology), DIF demonstrated deposition of IgG and C3 along the basement membrane zone, salt-split skin showed IgG deposition in the epidermal side of the blister, and immunoblotting showed reactivity against BP180. The occurrence of two autoimmune blistering conditions in the same patient is a rare event, and may suggest an intermolecular epitope-spreading phenomenon.

Anti-Bacterial Agents↗
PubMed2003

The role of intramolecular epitope spreading in the pathogenesis of endemic pemphigus foliaceus (fogo selvagem).

We report here a relationship between intramolecular epitope spreading and the clinical onset of the endemic form of pemphigus foliaceus in a Brazilian community with a high prevalence and incidence of the disease. Also known as Fogo Selvagem (FS), this disease is characterized by severe skin blistering and pathogenic anti-desmoglein-1 (Dsg1) autoantibodies. These autoantibodies bind the Dsg1 ectodomain and trigger keratinocyte cell detachment, the hallmark of FS. We show that (a) sera from FS patients in the preclinical stage recognized epitopes on the COOH-terminal EC5 domain of Dsg1, (b) disease onset was associated with the emergence of antibodies specific for epitopes on the NH2-terminal EC1 and EC2 domains, (c) all sera from FS patients with active disease recognized the EC1 and/or EC2 domains, and (d) sera from FS patients in remission showed reactivity restricted to EC5. These results suggest that anti-Dsg1 autoantibodies in FS are initially raised against the COOH-terminal EC5 domain of Dsg1 in individuals without skin disease; in genetically predisposed subjects the autoimmune response may then undergo intramolecular epitope spreading toward epitopes on the NH2-terminal EC1 and EC2 domains of Dsg1 leading to disease onset. Moreover, intramolecular epitope spreading may also modulate remissions and relapses of FS.

Antibody Specificity↗
PubMed1992

cDNA cloning of a novel human ubiquitin carrier protein. An antigenic domain specifically recognized by endemic pemphigus foliaceus autoantibodies is encoded in a secondary reading frame of this human epidermal transcript.

Autoantibodies from a patient suffering from endemic pemphigus foliaceus (EPF), a blistering skin disease, were used to screen a lambda gt11 human keratinocyte cDNA library. One immunoreactive cDNA clone (lambda EPF5) containing a 900-base pair insert was isolated and subjected to further analysis. Eight of 25 EPF sera were shown to react with the EPF5 fusion protein on immunoblots. The EPF5 cDNA insert hybridized with a 1.2-kilobase epidermal RNA transcript on a Northern blot. Sequence analysis revealed that lambda EPF5 contained the complete coding sequence for a 24-kDa polypeptide exhibiting significant sequence homology with a family of enzymes known as ubiquitin carrier proteins, or E2s, which are an essential component of the ubiquitin-protein conjugation system. The homology was particularly high in the core region containing the active site cysteine. The keratinocyte ubiquitin carrier protein expressed in bacteria, and isolated either intact or as a glutathione S-transferase fusion protein, exhibited the ability to form a thiol ester linkage with ubiquitin in a ubiquitin activating enzyme (E1)-dependent manner, a characteristic property of ubiquitin carrier proteins. The E2 enzyme encoded by clone EPF5 is the first member of this protein family to be cloned from an epidermal source. Interestingly, the EPF autoantibody-reactive epitope and the ubiquitin carrier protein were shown to be encoded in two different translational reading frames. The relevance of the cloned EPF epitope in the pathogenesis of this autoimmune disorder remains to be determined.

Amino Acid Sequence↗
PubMed2004

Detection of circulating autoantibodies using living keratinocyte staining on MCA-B1 method in dogs with pemphigus foliaceus.

In this study, we compared the sensitivity and specificity of three immunofluorescence techniques used to detect circulating autoantibodies in dogs with pemphigus foliaceus (PF); living keratinocyte staining on a canine keratinocyte cell line, MCA-B1, indirect immunofluorescence (IIF) on canine lip and IIF on bovine esophagus. Sera from canine PF cases were positive in four out of 27 dogs (14.8%) using living keratinocyte staining on MCA-B1 cells method, and five (18.5%) and eight sera (29.6%) using IIF on canine lip and bovine esophagus methods, respectively. By contrast, none of the 31 sera from dogs with non-pemphigus dermatoses reacted with MCA-B1 cells, whereas two (6.5%) as well as five sera (16.1%) obtained from those dogs showed positive reactivity with IIF on canine lip and bovine esophagus, respectively. Our results suggest that, although it exhibits the least sensitivity, the positive reactivity obtained by living keratinocyte staining on MCA-B1 cells can support the diagnosis of canine PF.

Animals↗
PubMed2004

Pemphigus foliaceus: an adverse reaction to lisinopril.

Lisinopril is an angiotensin converting enzyme inhibitor used in the treatment of cardiovascular disease. We report a case of pemphigus foliaceus in a 66-year-old male treated with lisinopril for hypertension and a previous myocardial infarction. The drug-induced variant of pemphigus is caused by a wide variety of drugs and is most frequently associated with captopril and penicillamine. It has not previously been reported in this commonly used drug.

Aged↗
PubMed1998

Juvenile pemphigus foliaceus.

A 7-year-old girl with generalized erythematous, scaling plaques and vesiculobullous lesions on the extremities was diagnosed as having pemphigus foliaceus. Lesional direct immunofluorescence revealed intercellular IgG, IgA and C3 deposition. The patient's serum gave positive reactions against one epitope of desmoglein 3 and the epitope of desmoglein 1 in enzyme-linked immunosorbent assays, but the blood sample for indirect immunofluorescence did not display any circulating antibodies. The patient was successfully treated systemically with prednisolone and dapsone. Currently, she is taking dapsone, 12.5 mg daily. She has been free of lesions for the last 3 years.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2002

Putative drug-related pemphigus foliaceus in four dogs.

Four dogs developed cutaneous lesions following the administration of various antibiotics. Histopathology of the lesions was compatible with pemphigus foliaceus, although apoptotic cells suggestive of erythema multiforme were seen in two cases. In two dogs the lesions resolved after 7.5-8.5 months of immune-suppressive treatment. No recurrence was seen during the follow-up period (3 and 4.5 years). The lesions in the other two dogs resolved within 3 weeks to 3 months following discontinuation of the antibiotic. No recurrence of clinical signs occurred during the follow-up period (1 and 4 years, respectively).

Animals↗
PubMed2002

Ultrastructural aspects of mucosas in endemic pemphigus foliaceus.

OBJECTIVE: To investigate whether ultrastructural changes present in clinically normal oral mucosa could occur in the mucosas of patients with endemic pemphigus foliaceus (EPF) or fogo selvagem (wildfire). PATIENTS: Surgical biopsy specimens were taken from the foreskin of 8 patients with EPF and 3 control subjects, the uterine cervix and vaginal wall of 9 patients with EPF and 2 controls, and the oral mucosa of 5 patients with EPF and 4 controls. The patients received a clinical and histopathologic diagnosis of EPF and all had clinically normal oral and genital mucosas. RESULTS: In the patients with EPF, widening of the intercellular spaces and distended, elongated cytoplasmic projections, the tips of which contained desmosomes and were sometimes disassembled, were evident in all 4 regions studied. At the periphery of the spinous cells, cytoplasmic vesicles apparently containing intact or fragments of desmosomes or half-desmosomes were seen. CONCLUSIONS: The ultrastructural lesions found in the mucosas studied are similar to those previously described in the literature for the oral mucosa of patients with EPF. In the cases of EPF, even though the desmosomal changes occurred in all epithelial layers, blisters did not occur in the mucosas by possible coexpression of desmoglein 1 and desmoglein 3.

Adult↗
PubMed2003

Prevalence of autoantibodies in patients with endemic pemphigus foliaceus (fogo selvagem).

OBJECTIVE: The aim of the present study was to investigate a broad spectrum of autoantibodies in patients with endemic pemphigus foliaceus (EPF)-fogo selvagem-and to determine the possible association between EPF and other autoimmune diseases. MATERIALS AND METHODS: Indirect immunofluorescence was used to test 120 patients with EPF and 200 healthy controls for the presence of the following autoantibodies: anti-desmoglein-1 (APF), anti-neutrophil cytoplasmic (ANCA), anti-smooth muscle (SMA), anti-mitochondrial (AMA), anti-nuclear (ANA), anti-liver kidney microsomal (LKM), anti-gastric parietal cells (GPCA) and anti-thyroid microsome (TMA). RESULTS: APF antibodies were detected in 62.5% of the patients (75/120), ANA and SMA in 0.8% (1/120), and TMA in 1.6% (2/120). None of the patients was positive for ANCA, AMA, LKM or GPCA. In the control group, a positivity of 2% was observed for SMA (4/200), 1.5% for TMA (3/200), and 0.5% (1/200) for ANA and GPCA. None of the controls was positive for APF, LKM, AMA or ANCA. CONCLUSIONS: The prevalence of the autoantibodies ANA, SMA, AMA, GPCA, LKM and ANCA in patients with EPF was similar to that observed in the control group. No association with clinical or laboratory manifestations of other concomitant autoimmune diseases was observed in EPF patients. These results confirm the concept that EPF is an organ-specific autoimmune disease.

Adolescent↗
PubMed1990

Pemphigus foliaceus antigen: characterization of an immunoreactive tryptic fragment from BALB/c mouse epidermis recognized by all patients' sera and major autoantibody subclasses.

The pemphigus foliaceus antigen (PF Ag) is a 160-kDa desmosomal core glycoprotein, desmoglein I. A 50-kDa soluble immunoreactive fragment of the PF Ag was recently prepared from trypsinized cornified cell envelope preparations by papain treatment (R.S. Labib et al. 1989, J. Invest. Dermatol. 93, 272-279). This papain fragment (pf-PF) is associated with upper cell layers of the epidermis and appears to be trypsin resistant in situ. The present work describes the preparation of another fragment by trysinization of the viable lower cells of the epidermis of neonatal BALB/c mice. This tryptic fragment (tf-PF) is a 45-kDa glycoprotein that is partially purified by concanavalin A affinity chromatography of the trypsinization medium. The partially purified tf-PF preparation is capable of completely blocking the indirect immunofluorescence of high titer PF sera. The tf-PF is immunoprecipitated by all PF sera tested (n = 19) and by the two major subclasses of PF autoantibodies, IgG1 and IgG4. Autoantibodies of both the predominant IgG4 and the less prevalent IgG1 subclasses precipitate the same tf-PF as demonstrated by a single compact spot of pI 5.5 by two-dimensional polyacrylamide gel electrophoresis. Chemical and immunological comparison of the tf-PF and pf-PF may explain why the acantholytic lesions of PF appear only in the upper epidermis, despite the presence of the PF Ag throughout all layers of the epidermis. The availability of these two soluble immunoreactive fragments of the PF Ag will be of great value for the further immunochemical characterization of the antigenic epitopes and their role in cell-cell adhesion.

Animals↗
PubMed1994

[Pemphigus foliaceus in a foal. A case history].

The clinical history, clinical, pathological and immunohistological findings of a four-month-old foal with generalised pemphigus foliaceus are presented. The typical lesions of this autoimmune skin disease are described and discussed.

Animals↗
PubMed1991

The calcium-sensitive epitope of pemphigus foliaceus antigen is present on a murine tryptic fragment and constitutes a major antigenic region for human autoantibodies.

Recent findings indicate that the pemphigus foliaceus (PF) antigen is involved in epidermal cell adhesion and that characteristic PF lesions result from loss of this function as a consequence of autoantibody binding. In the present communication we present data on the epitopes involved in the human autoantibody binding to an immunologically reactive murine tryptic fragment of the PF antigen (tf-PF). Immunoprecipitation experiments showed that 39 PF sera, obtained from North American, Colombian, and Brazilian patients recognized only calcium-sensitive epitope(s) on the tf-PF. Immunofluorescence blocking experiments showed that preincubation with tf-PF completely blocked the immunofluorescence of 80% of the sera when tested on human skin substrate, and 86% of the sera when tested on murine skin substrate. These results show that the calcium-sensitive epitope(s) originally recognized on human PF complex, is (are) present on the murine tf-PF and constitute(s) a major antigenic region for the human PF autoantibodies. They also implicate this region of the PF antigen in the pathogenesis of PF as well as in epidermal cell adhesion.

Animals↗