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At least 91 records · Page 5Linked to original sources
PubMed1995

Pemphigus vulgaris and pemphigus foliaceus sera show an inversely graded binding pattern to extracellular regions of desmosomes in different layers of human epidermis.

We analyzed the location of binding sites for pemphigus vulgaris (PV) antigen and pemphigus foliaceus (PF) antigen in the human epidermis using serum samples obtained from three patients with PV and three patients with PF. Confocal laser scanning microscopy, immunofluorescent examination of ultrathin cryosections, and immunoperoxidase electron microscopy demonstrated discontinuous dots along the epidermal cell surfaces. Immunogold electron microscopy of ultrathin cryosections showed specific binding of PV and PF autoantibodies only to desmosomes. Post-embedding immunogold electron microscopy using cryofixation and cryosubstitution enabled the whole depth of the epidermis to be examined and the binding of PV and PF autoantibodies to be quantitated by counting gold particles. Both PV and PF autoantibodies bound to all desmosomes in the epidermis, but not to the surface of the non-desmosomal keratinocytes. The majority of auto-antibody binding occurred in the extracellular domain (PV, 62%; PF, 69%). The statistical analysis of two-way analysis of variance regarding the number of gold particles labeling a single desmosome confirmed a significant interaction between subtypes of pemphigus (PV and PF) and the different epidermal cell layers (p < 0.044). The results indicate that the number of gold particles bound to individual desmosomes with PV sera was significantly higher in the lower epidermis than in the upper epidermis, and that of PF sera showed reciprocal pattern. This inversely graded binding pattern suggests heterogeneity of the composition of the desmosomes, which may explain the differences in level of acantholysis between PV and PF.

Autoantibodies↗
PubMed1977

Characterization of cryoprecipitates in pemphigus: demonstration of pemphigus antibody activity in cryoprecipitates using the immunofluorescent technique.

Cryoproteins observed in patients with pemphigus have been shown to contain IgG, complement components, and other serum proteins. The IgG moiety possessed pemphigus antibody activity and contained kappa and lambda light chains. Cryoproteins appeared in the clinically active stage and disappeared subsequent to clinical improvement. These observations indicate that immune complexes may exist in the sera of patients with pemphigus.

Adult↗
PubMed1982

Neonatal pemphigus vulgaris: role of passively transferred pemphigus antibodies.

A neonate with oral lesions of pemphigus vulgaris is described. The diagnosis was confirmed by cytological examination of lesions, positive intercellular IgG staining of the acantholytic cells, and presence of pemphigus antibodies in his serum. At the time of delivery, the child's mother had active pemphigus vulgaris, and it is suggested that this was a case of passively transferred disease.

Adolescent↗
PubMed1991

Ultrastructural binding site of pemphigus foliaceus autoantibodies: comparison with pemphigus vulgaris.

We studied in vivo binding sites of pemphigus foliaceus (PF) autoantibodies by immuno-gold labelling technique, and compared them with those of pemphigus vulgaris (PV). In early acantholytic lesions of PF, the bound antibodies indicated by 5 nm protein A-colloidal gold particles were observed on the surface of keratinocytes, with particular affinity for desmosomes and separated attachment plaques. Nondesmosomal cell surfaces were sparsely labeled with the gold particles. A similar binding pattern was seen in the epidermal sheets obtained from a PV patient utilizing the Nikolsky phenomenon. These findings indicate that both PF and PV antigen-antibody complexes are densely located on the desmosomal areas in early pemphigus lesions, suggesting the pathogenic importance of functional impairment of desmosomes by the autoantibodies.

Aged↗
PubMed1994

Shift in clinical features, histologic findings and antigen profiles from pemphigus vulgaris to pemphigus foliaceus--two case studies.

We investigated antigen profiles of the sera from 2 cases of pemphigus vulgaris (PV) who subsequently developed pemphigus foliaceus (PF). Immunoblotting was carried out using normal human epidermal extracts as the antigen source. The sera during PV reacted exclusively with the 130-kD PV antigen and the sera during PF exclusively with the 150-kD antigen. Therefore, dermatologic, histologic and immunohistologic findings before and after this transition were clearly shown related to changes in serum reactivity with pemphigus antigens.

Autoantigens↗
PubMed1998

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 to eliminate the ability of PNP sera to induce cutaneous blisters in neonatal mice in vivo. Furthermore, anti-Dsg3-specific antibodies that were affinity purified from PNP sera were proven to be pathogenic and caused blisters in neonatal mice. These findings indicate that Dsg3 and Dsg1 are the cell surface target antigens in PNP and that IgG autoantibodies against Dsg3 in PNP sera play a pathogenic role in inducing loss of cell adhesion of keratinocytes and causing blister formation.

Acantholysis↗
PubMed2005

The interaction of pemphigus autoimmunoglobulins with epidermal cells: activation of the fas apoptotic pathway and the use of caspase activity for pathogenicity tests of pemphigus patients.

Pemphigus is a fatal autoimmune disease in which autoimmunoglobulins PV-IgG (binding to desmoglein 3) and PF-IgG (binding to desmoglein 1) in pemphigus vulgaris and pemphigus foliaceus, respectively, cause intraepidermal blisters, cell-cell separation (acantholysis), and cell death. The mechanism of acantholytic lesion formation has not yet been elucidated. Recently, we have reported that an apoptotic mechanism might be operative in PV-IgG-induced acantholysis: (1) in patients' lesional and some perilesional skin portions, the FasR pathway is activated as its components were enriched; (2) in cultured keratinocytes, PV-IgG upregulates effectors of the FasR pathway (including the mitochondrial loop), as found by immunodetermination (cytochemistry, Western blot of pathway effectors) and determination of caspases 1, 3, and 8 activity/activation; (3) in organ cultures of skin incubated with PV-IgG, activated caspase 8 was found also in perilesional cells and coaggregated with bound PV-IgG; (4) caspase 8 activation in DISCs precedes caspase 3 activation in keratinocytes in cultures upon incubation with PV-IgG. Because caspase activation was shown to accompany lesion formation in cell and organ cultures incubated with PV-IgG, we used caspase activity to monitor the pathogenicity of PV-IgG in relation to PV-IgG binding to epithelia. A rough correlation was found between sera titers, determined by IIF and by immunoblot binding to desmoglein 3, and activation of caspase 3.

Acantholysis↗
PubMed2006

Transition from pemphigus foliaceus to pemphigus vulgaris: case report with literature review.

The transition between the main subtypes of pemphigus, pemphigus vulgaris (PV), and pemphigus foliaceus (PF) has rarely been reported. Moreover, the development of PV in a patient with PF is much more unusual than that of PF in a patient with PV. We report a 48-year-old man who presented with cutaneous lesions showing the typical clinical and histological features of PF. Five years later, his skin lesions became extensive and he developed oral erosions. His condition did not respond well to steroids and azathioprine. Histological examination of a vesicle disclosed suprabasal acantholysis in contrast to the subcorneal acantholysis discovered upon initial histological evaluation. Indirect immunofluorescence revealed IgG antikeratinocyte cell surface antibodies at a titer of 1:640. The titer was 1:160 at initial diagnosis. Upon immunoblotting, the patient's serum reacted with 130 kiloDalton (kDa) and 160 kDa proteins, suggesting desmoglein (Dsg) 3 and 1, respectively. We herein report an unusual case of PV that developed from PF during the disease's flare-up.

Adult↗
PubMed1989

[Pemphigus vegetans, a rare variant of pemphigus vulgaris].

Pemphigus vegetans is a variant of pemphigus vulgaris with different clinical manifestations. Diagnosis may be difficult because of the varied presentations. A 46-year-old woman with this condition had been treated for Behcet's disease because of oral lesions. On admission, prior to dermatological consultation, she was thought to have an infectious disease. The diagnosis of pemphigus vegetans was confirmed by histological and immunofluorescence studies.

Diagnosis, Differential↗
PubMed1987

[Pemphigus vegetans of the Hallopeau type. Detection of pemphigus antibodies with direct and indirect immunofluorescence].

We report the case of a 25-year-old woman with bullous lesions diagnosed as the rare pustular type of pemphigus described by Hallopeau. Direct immunofluorescence studies revealed deposits of IgG and C3 in the intercellular spaces of the epidermis; the indirect immunofluorescence technique revealed circulating antiepithelial IgG antibodies. The skin lesions cleared completely following treatment with systemic corticosteroids and azathioprine. These findings indicate the disease first described by Hallopeau as "pyodermite végétante" does indeed belong to the pemphigus group, being a rare type of pemphigus vegetans.

Adult↗
PubMed1979

In vivo bound pemphigus antibodies in a stillborn infant. Passive intrauterine transfer of pemphigus vulgaris?

Pemphigus vulgaris developed in a 23-year-old woman during the first trimester of pregnancy. In the 33d week she had a stillbirth of a female child who showed extensive skin lesions clinically suggestive of pemphigus. The application of direct immunofluorescence staining to skin tissue sections showed deposition of IgG in the intercellular spaces of the epidermis of both mother and child. Our findings raise the possibility of transplacentar passage of pemphigus antibody, which may have been responsible for the fetal skin lesions.

Adult↗
PubMedDate not supplied

Levels of serum IgE in patients with Brazilian pemphigus foliaceus and pemphigus vulgaris.

The level of serum IgE in thirty patients with pemphigus (19 with Brazilian pemphigus foliaceus (BPF) and 11 with pemphigus vulgaris (PV)) were determined by means of a solid phase radioimmunoassay. A significant (P less than 0.01) increase in the level of IgE was observed in BPF patients compared to the level of IgE in PV patients and healthy adults. The possible relationship of an elevated level of IgE with respect to other aberrations of immunologic responsiveness and the suggested infectious etiology of BPF are considered.

Humans↗
PubMed1980

[Pemphigus herpetiformis: a clinical variant of pemphigus vulgaris].

Two female patients are reported in whom the clinical picture closely corresponded to dermatitis herpetiformis Duhring or bullous pemphigoid, while acantholytic changes were present histologically. Indirect immunofluorescence revealed circulating pemphigus antibodies. Direct fluorescence showed deposits of IgG and complement in the intercellular space of the affected areas. One of the two patients responded well to DDS. There have been reports in the recent literature on bullous dermatoses, which can be regarded as morphological variants of pemphigus vulgaris. The term pemphigus herpetiformis is used here to designate these variants. The diagnosis can only be made by histological and immunological examinations in connection with the clinical picture.

Aged↗
PubMed1994

Brazilian pemphigus foliaceus, endemic pemphigus foliaceus, or fogo selvagem (wild fire).

Brazilian pemphigus foliaceus, also known as fogo selvagem, is the endemic form of pemphigus foliaceus occurring in certain regions of Brazil. Epidemiologic data strongly support the notion that this disease is caused by exposure to an environmental agent or agents. This form of pemphigus foliaceus is a true human autoimmune disease mediated by autoantibodies of the IgG class, IgG4 subclass. These autoantibodies are pathogenic and highly specific for the disease. The environment agent may sensitize the patient to produce autoantibodies. These autoantibodies may cross-react with the epidermis and induce disease in the patient.

Autoantibodies↗
PubMed1995

Immunofluorescence and immunoblot studies on the reactivity of pemphigus vulgaris and pemphigus foliaceus sera with desmoglein 3 and desmoglein 1.

We have investigated expression of pemphigus vulgaris (PV) antigen, desmoglein 3 (Dsg3), and pemphigus foliaceus (PF) antigen, desmoglein 1 (Dsg1), in various tissues. Immunofluorescence studies suggested that Dsg1 and Dsg3 are preferentially expressed in the upper and lower epidermis, respectively. With immunoblotting of human epidermal extracts, all PV sera reacted with Dsg3 but not with Dsg1. By contrast, only half of PF sera reacted with Dsg1 but none reacted with Dsg3. These results confirm the distinct antibody specificity between PV and PF sera. They also suggest that PV sera contain antibodies against linear epitopes present even on the denatured antigen, while some PF sera contain antibodies only against conformational epitopes present on the native antigen. With immunoblotting of bovine desmosome preparations, certain PV sera and an anti-Dsg monoclonal antibody reacted with both Dsg1 and Dsg3. Affinity-purification of these PV antibodies suggested that the simultaneous reactivity with Dsg1 and Dsg3 was produced by two different subsets of antibodies and not by cross-reactivity of single antibodies. This study indicates that pemphigus serum is a useful probe with which we learn various aspects of keratinocyte biology.

Animals↗
PubMed1997

The location of binding sites of pemphigus vulgaris and pemphigus foliaceus autoantibodies: a post-embedding immunoelectron microscopic study.

Pemphigus is a life-threatening autoimmune blistering disease of skin and mucous membranes that has two major subtypes based on clinical and histological features, pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Autoantibodies against the PV antigen (desmoglein 3) and the PF antigen (desmoglein 1) are involved in the pathogenesis of blister formation. In the present study, the location of epitopes recognized by autoantibodies of patients with PV and PF was studied by postembedding immunogold electron microscopy. PV and PF autoantibodies were observed bound predominantly to the intercellular domains of desmosomes, but not to the non-desmosomal keratinocyte cell surface. The relationship between the location of PF antigen and other constitutive desmosomal proteins, desmocollin, desmoplakin and plakoglobin, in normal human skin was investigated using a double immunogold labelling technique. It was observed that PF antigen and desmocollin co-localize within the intercellular domain of the desmosomes. In contrast, the antibodies against desmoplakin and plakoglobin bound predominantly to the intracellular desmosomal attachment plaque with the binding site of the antibody against plakoglobin closer to the desmosomal cell membrane than that of the antibody to desmoplakin. We show that the LR White postembedded immunogold electronmicroscopy technique is convenient and easily applied to studies of autoimmune bullous skin diseases. We have used it to demonstrate the precise localization of the binding sites of PV and PF autoantibodies and their relationship with other constitutive desmosomal proteins.

Antigens↗
PubMed1997

Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3)

We report a patient with herpetiform pemphigus (HP) who showed reactivity only with pemphigus vulgaris (PV) antigen but not with pemphigus foliaceus (PF) antigen. Direct and indirect immunofluorescence revealed keratinocyte cell surface staining in the lower layers of the epidermis, where desmoglein 3 (Dsg3) is expressed. Immunoblot analysis, using ethylenediamine tetra-acetic acid-separated human epidermal extracts, revealed that the patient's serum recognized only a 130-kDa polypeptide which co-migrated with Dsg3. By antigen-specific immunoadsorption studies, using desmoglein 1 (Dsg1) and Dsg3 recombinant protein produced by baculovirus expression system, immunoreactivity of the patient's serum was completely adsorbed by Dsg3 alone, but not by Dsg1. These results indicate that this HP patient produced only anti-Dsg3 autoantibodies and no other autoantibodies against components of the keratinocyte cell surface. HP could be a variant of PV, in addition to PF, with unique clinical and histological features.

Autoantigens↗
PubMed1998

Diet and pemphigus. In pursuit of exogenous factors in pemphigus and fogo selvagem.

Individuals with a genetic predisposition to pemphigus will develop the disease only when one or more additional factors are present. The nature of these factors is as yet unknown, but our starting point was that certain drugs (penicillamine, captopril, and rifampicin) are recognized as such factors. Since some nutrients have chemical compositions similar to these known causative drugs, these nutrients may act similarly and, therefore, nutritional factors should also be suspected. As when drugs are involved, elimination of the inciting ingredients may be crucial for management of the disease. This article discusses the possible role of nutritional ingredients in the disease process of pemphigus, including fruit, leaves, roots, seeds, and even water. Possible causative candidates are thiol, thiocyanate, phenols, and tannins.

Diet↗