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At least 19 recordsLinked to original sources
PubMed1991

Drug-induced pemphigus: autoantibodies directed against the pemphigus antigen complexes are present in penicillamine and captopril-induced pemphigus.

Pemphigus is an autoimmune blistering disease characterized by circulating autoantibodies directed against the keratinocyte cell surface. The two variants, pemphigus foliaceus and pemphigus vulgaris, can be distinguished at the molecular level by immunochemical studies. The large majority of patients with pemphigus develop the disease spontaneously; however, there is a small group of patients who develop pemphigus after treatment with certain medications, of which penicillamine and captopril are the best documented. Most patients with drug-induced pemphigus have circulating and/or tissue bound epidermal cell surface autoantibodies; however, the molecular specificity of these autoantibodies has not been studied. We performed immunoprecipitation studies utilizing extracts of 125I-labeled suction blister epidermis and the sera of three patients with drug-induced pemphigus foliaceus (two due to penicillamine and one due to captopril) and one patient with captopril-induced pemphigus vulgaris. We found that the three patients with drug-induced pemphigus foliaceus had circulating autoantibodies that are directed against the pemphigus foliaceus antigen complex and that the one patient with drug-induced pemphigus vulgaris had circulating autoantibodies that are directed against the pemphigus vulgaris antigen complex. This study demonstrates that autoantibodies from drug-induced pemphigus patients have the same antigenic specificity, on a molecular level, as do autoantibodies from other pemphigus patients.

Aged↗
PubMed1991

Regional variation in the expression of pemphigus foliaceus, pemphigus erythematosus, and pemphigus vulgaris antigens in human skin.

The expression of the pemphigus foliaceus (PF), pemphigus erythematosus (PE), and pemphigus vulgaris (PV) antigens in 16 different regions of normal human skin was evaluated by indirect immunofluorescence by using sera with a high titer of PF, PE, and PV antibodies. Regional variations were observed in the expression of all these antigens. The expression of the PF and PE antigens, as measured by endpoint titer of antibody reactivity, was highest in skin specimens obtained from the upper torso, and lowest in those from the buccal mucosa, lower torso, and scalp. This distribution pattern differed from that of PV antigen, whose expression was highest in buccal mucosa and scalp. These patterns correlate with, and may provide a partial explanation for, the different distribution of skin lesions in these different forms of pemphigus.

Antigens, Surface↗
PubMed1991

IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis.

A patient with clinical features of herpetiform pemphigus of the foliaceus type had histologic findings consistent with pemphigus. Intercellular IgA deposits in a pattern like that of IgG in pemphigus were present. Circulating pemphigus-type IgA-class antibodies reacted first only with guinea pig and later in the disease also with monkey esophagus sections. IgG-class pemphigus antibodies were blocked by the IgA-class antibodies of this patient. In addition, the IgA-class pemphigus antibodies in this patient were blocked by the IgG-class pemphigus antibodies in tests on guinea pig and monkey esophagus. This indicates that the IgA-class antibodies in this patient were directed either to the same antigen as the IgG-class pemphigus foliaceus antibodies or to one that is close enough to it to give steric hindrance. The skin lesions responded poorly to systemic corticosteroid therapy. Dapsone therapy initially produced dramatic improvement, but the condition flared to the point that plasmapheresis, in addition to high doses of corticosteroids and cyclophosphamide, had to be used to control it.

Acantholysis↗
PubMed1991

Desmoplakin I and II in acantholytic dermatoses: preservation in pemphigus vulgaris and pemphigus erythematosus and dissolution in Hailey-Hailey's disease and Darier's disease.

Desmoplakin I and II are important components of the attachment plaque of the desmosome which mediates cell to cell adhesion, in epithelial cells. In this study we used well-characterized antibody against desmoplakin I and II immunohistochemically and immunoelectron microscopically on two cases of pemphigus vulgaris and one case of pemphigus erythematosus and two cases each of Hailey-Hailey's disease and Darier's disease. In the normal human epidermis the desmosomes were demonstrated in a dotted pattern along cell periphery. In pemphigus vulgaris and pemphigus erythematosus acantholytic cells and the perilesional cells exhibited normal dotted pattern along the cell periphery. In Hailey-Hailey's disease and Darier's disease, the dotted pattern is lost in acantholysed and perilesional areas and anti-desmoplakin I + II positive proteins were observed diffusely in the cytoplasm. Immunoelectron microscopical findings correspond to these light microscopical observations. It is concluded that in autoimmune acantholytic disease such as pemphigus vulgaris and pemphigus erythematosus, desmoplakins are intact even in acantholytic cells, whereas in genodermatoses such as vulgaris and pemphigus erythematosus, desmoplakins are intact even in acantholytic cells, whereas in genodermatoses such as Hailey-Hailey's disease and Darier's disease primary or secondary abnormalities abnormalities of desmosomes may be involved in their pathogenesis.

Acantholysis↗
PubMed1978

Fate of pemphigus antibody following successful therapy. Preliminary evaluation of pemphigus antibody determinations to regulate therapy.

Six of 17 pemphigus patients (35%) treated during a six-year period with immunosuppressive agents and/or corticosteroids have had prolonged clinical and immunologic remissions off all therapy. All were treated until serum and tissue bound pemphigus antibodies could no longer be detected. The length of remission has ranged from 1 1/2 years to 4 years. Three of these six patients relapsed after being clinically and serologically free of pemphigus for 19, 20, and 48 months. Seven additional patients are clinically free of disease with insignificant pemphigus antibody titers of 10 or less. Therapy now is being discontinued gradually in these patients. This preliminary study demonstrates that (1) a large percentage of pemphigus patients may have a prolonged clinical and immunological remission after successful therapy; (2) maintenance therapy may not be required to preserve the remission; (3) monitoring serum and in vivo bound pemphigus antibody is of value in regulating therapy in pemphigus patients.

Adrenal Cortex Hormones↗
PubMed1992

Pemphigus vulgaris in siblings: HLA-DR4 and HLA-DQw3 and susceptibility to pemphigus.

BACKGROUND: The association of pemphigus vulgaris with the HLA serotypes, DR4 and DRw6, and with the DQ-beta chain alleles, DQw1 and DQw3, suggests that there is a genetic predisposition to this disease. However, familial cases of pemphigus vulgaris are exceedingly rare. OBJECTIVE: We studied two siblings with pemphigus vulgaris and three unaffected family members to determine whether an HLA allele was associated with the development of pemphigus vulgaris in this family. METHODS: We utilized restriction fragment length polymorphism methods using the HLA-DR beta 1, HLA-DQ alpha, and HLA-DW beta 1 genes as cDNA probes. RESULTS: We found that the affected siblings share the haplotype HLA-DR4 and the DQw.3.2 allele. CONCLUSION: Our findings of gene sharing among siblings with pemphigus vulgaris lend support to previous studies of unrelated Caucasian patients, which implicated DQw3 allele polymorphisms in conferring susceptibility to pemphigus.

Adult↗
PubMed1991

Can pemphigus vulgaris become pemphigus foliaceus?

Among 31 patients with pemphigus, we observed two women with clinical and histologic features characteristic of pemphigus vulgaris that later became those of pemphigus foliaceus. Western blot study indicated that serum from one patient, when she had the cutaneous manifestations of pemphigus foliaceus, contained antibodies reactive with a desmosomal antigen, suggestive of desmoglein 1, the same as recognized by pemphigus foliaceus serum. The present study suggests that two distinct types of pemphigus can occur in the same person simultaneously or separately.

Acantholysis↗
PubMed1979

Appearance of "pemphigus acantholysis factor" in human skin cultured with pemphigus antibody.

These studies deal with the mechanism of pemphigus IgG-induced epidermal acantholysis. When normal human skin was culted with defined medium containing IgG from pemphigus serum, extensive epidermal acantholysis developed and heat-labile proteolytic enzyme(s) were recovered in the culture medium. The enzyme(s) displayed maximal activity at pH 6.5 when a 3H-amino acid-labeled, insoluble epidermal cell material was used as substrate. The enzyme activity increased during the first 3 days of culture and the appearance of maximal activity coincided with the time of onset of acantholysis. Acantholysis did not occur in control cultures incubated with normal IgG and the enzyme did not appear in the medium or in aqueous extracts of cultured tissues. The enzyme(s) is probably not of lysosomal origin because low pH-active proteases characteristic of these organelles remained within the cells. The effects of puromycin on appearance of enzyme activity, acantholysis and cell viability was studied. At cytotoxic concentrations, the appearance of the enzyme(s) and acantholysis were prevented, whereas at less toxic concentrations enzyme activity and acantholysis were not prevented. Because inhibition of protein synthetic rates by puromycin could not be dissociated from the cytotoxic effects, it is uncertain whether enzyme appearance and acantholysis were dependent upon living tissue or on specific protein synthesis. After pemphigus IgG was removed from the conditioned medium by DEAE cellulose and affinity column chromatography, the remaining material contained enzyme activity and caused acantholysis in fresh skin explants. Similar activities were not present in normal IgG-containing conditioned medium or unfractionated epidermal extracts from normal skin. These data indicate that when the pemphigus IgG autoantibody interacts with epidermal cell surface antigens, the cell responds by synthesis or activation of a non-IgG "pemphigus acantholysis factor" (PAF) which may be a nonlysosomal proteolytic enzyme. It is suggested that PAF causes loss of adhesion between keratinocytes and ultimately produces the characteristic acantholytic cells of pemphigus.

Acantholysis↗
PubMed1991

Coexistence of pemphigus foliaceus and bullous pemphigoid. Demonstration of autoantibodies that bind to both the pemphigus foliaceus antigen complex and the bullous pemphigoid antigen.

Pemphigus and bullous pemphigoid are autoimmune blistering diseases of the skin characterized by circulating autoantibodies directed against the keratinocyte cell surface and the epidermal basement membrane zone, respectively. The coexistence of pemphigus and bullous pemphigoid is very uncommon. We describe a patient with pemphigus foliaceus who later developed bullous pemphigoid and show, by means of immunoprecipitation studies utilizing both cultured keratinocytes and suction blister epidermis, that our patient had circulating autoantibodies directed against both the pemphigus foliaceus antigen complex and the bullous pemphigoid antigen. This report is the first to demonstrate the coexistence of pemphigus foliaceus and bullous pemphigoid at the molecular level.

Antigen-Antibody Complex↗
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↗
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↗
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↗
PubMed1977

Organ culture studies of pemphigus antibodies. II. Ultrastructural comparison between acantholytic changes in vitro and human pemphigus lesions.

The ultrastructural and light microscopic features of acantholysis produced in organ culture were compared with those of human pemphigus lesions. In both, an intraepidermal split was seen an typical suprabasal acantholytic cells were present. These cells contained small bundles of tonofilaments, usually located away from the cell periphery. Desmonsomal plaques with inserted tonofilaments frequently remained along the periphery of acantholytic cells and along the upper portion of the periphery of basal cells. The ultrastructural similarity between in vitro and in vivo lesions provides additional evidence to suggest that organ cultures may provide a valid model for studying the dynamics of pemphigus lesion formation.

Aged↗
PubMed1991

Ultrastructural localization of pemphigus vulgaris and pemphigus foliaceus antigens in cultured human squamous carcinoma cells.

The ultrastructural localization of the pemphigus vulgaris (PV) and pemphigus foliaceus (PF) antigens in cultured human squamous carcinoma cells was observed using immunogold electron microscopy. Both the PV and PF autoantibodies bound only to the extracellular portion of the desmosomal structures. After incubation at 37 degrees C, the PV antigen-antibody complexes were observed within the cultured cells. PV and PF antigen expression was markedly reduced when the cells were cultured in medium with a low Ca2+ concentration.

Antigen-Antibody Complex↗
PubMed1975

Herpetiform pemphigus, a variable pattern of pemphigus.

A patient with the clinical features of dermatitis herpetiformis and the histological features of pemphigus, subcorneal dermatosis and dermatitis herpetiformis did not respond to treatment with pyridine or sulfones. Systemic steroids and azothioprine were effective. Immunofluorescence studies demonstrated circulating pemphigus antibodies and IgG bound in vivo in the intercellular spaces of the epidermis.

Abscess↗
PubMed1991

[Isolated pemphigus vulgaris of the mouth mucosa--pemphigus test: diagnostic confirmation using direct immunofluorescence study of normal skin].

The diagnosis of oral pemphigus vulgaris (PV) without concomitant cutaneous manifestations is often complicated by difficulties in obtaining a biopsy specimen allowing histological examination and direct immunofluorescence (IF) microscopy. In this clinical study five of our patients with oral PV were investigated: biopsies were taken both from lesional oral mucous membranes and from unaffected gluteal skin for IF analysis. Identical IF results, with intercellular epidermal deposition of IgG in all and of C3 in two of the five cases, were obtained in lesional and nonlesional biopsies. Therefore, it is concluded that with current IF techniques examination of the normal skin is sufficient for the diagnosis of oral PV; thus, the often painful and difficult removal of biopsy specimens from within the affected oral cavity can be avoided. Further studies are needed to confirm the sensitivity of this method.

Adult↗