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PubMed1992

Adhesion molecules and related proteins in Darier's disease and Hailey-Hailey disease.

We have used antibodies to plakoglobin and E-cadherin: the lectins, peanut agglutinin (PNA) and soybean agglutinin (SBA); and sera from patients with the autoimmune diseases pemphigus vulgaris (PV) or pemphigus foliaceus (PF), in an immunohistological study of Darier's disease and Hailey-Hailey disease. There was normal expression of plakoglobin, E-cadherin, lectins and pemphigus antigens at the periphery of keratinocytes in uninvolved skin. Clumps of plakoglobin were detected within acantholytic cells in Hailey-Hailey disease, whereas expression was diffuse in acantholytic cells in Darier's disease. This difference may reflect differences in the pathogenesis of acantholysis. E-cadherin expression was weak or absent at the periphery of some acantholytic cells; lectin binding was sometimes reduced around acantholytic cells, and pemphigus antibodies did not bind to the acantholytic cells involved skin in either disease. Internalization, conformational changes or proteolysis may alter the expression of extracellular epitopes by acantholytic cells.

Acantholysis↗
PubMed1994

Pemphigus in Oxford, UK, and New Delhi, India: a comparative study of disease characteristics and HLA antigens.

A study of pemphigus in New Delhi, India, and Oxford, UK, was undertaken including 20 patients in Oxford and 50 in New Delhi. Data included clinical and histological subtypes and socio-economic data; patients were HLA typed. In New Delhi pemphigus vulgaris predominated, but in Oxford pemphigus vulgaris and pemphigus foliaceus have equal prevalence. Disease distribution with sex was the same, but age at onset was significantly lower in New Delhi (p = 0.0019). HLA typing in pemphigus vulgaris patients revealed a significant reduction in HLA-DR2 in New Delhi (p = 0.0008) and Oxford (p = 0.09). A small increase in HLA-DR1 and -DR4 was found in both groups and, in males only, a subtle increase in HLA-DR6 and reduction in HLA-DR3. No differences were found in the class I antigens. Thus there are striking differences in the types of pemphigus between the two populations, yet the genetic predisposition is the same.

Adolescent↗
PubMed2003

Paraneoplastic pemphigus is associated with the DRB1*03 allele.

Pemphigus is a group of autoimmune blistering diseases caused by autoantibodies directed against keratinocyte adhesion molecules. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF), in which autoantibodies bind, respectively, to desmoglein 3 and desmoglein 1, are strongly associated with HLA-class II DR4 and DR14 alleles. In paraneoplastic pemphigus (PNP), a rare variant associated with neoplasia, autoantibodies target proteins of the plakin family in addition to desmogleins 1 and 3. The presence of anti-desmoglein antibodies in all types of pemphigus raises the question of common molecular mechanisms of susceptibility, particularly similar MHC-class II allele associations, in the different forms of the disease. HLA-DRB1 typing was performed in 13 PNP patients and results were compared to those obtained from 84 healthy controls, 37 PV and 31 PF patients. Our data demonstrate a significant association of PNP with HLA-DRB1*03 allele which was found in 61.5% of the patients, whereas DRB1*04 and DRB1*14 appear not to be involved in PNP susceptibility. Therefore, the HLA-genetic background of PNP differs from that of other types of pemphigus, which suggests that distinct mechanism(s) initiate(s) the immunological response in this form of pemphigus.

HLA-DR Antigens↗
PubMed1990

The clinical and histopathological spectrum of IgA-pemphigus--report of two cases.

Two new cases of neutrophilic bullous disease exhibiting bound and circulating intercellular IgA in vivo in both direct and indirect immunofluorescence (IF), for which the term IgA-pemphigus was recently proposed, are presented. The first case showed a unique constellation of clinical and histopathological features not previously described for IgA-pemphigus, resembling both dermatitis herpetiformis and pemphigus, for which the designation IgA-herpetiform pemphigus seems most appropriate. The second case showed clinical and histopathological features mimicking subcorneal pustular dermatosis for which the previously used term IgA-pemphigus foliaceus seems most appropriate. The previously reported 11 cases showing similar direct IF findings, as well as our two patients, illustrate the main differences between IgA-pemphigus and classic forms of this condition: (a) different clinical manifestations with the absence of Nikolsky's sign; (b) scanty acantholysis; an abundance of neutrophils, with occasional neutrophilic spongiosis; (c) absence of C3 on direct IF; (d) the lower sensitivity of indirect IF and low levels of circulating antibodies; (e) the relatively benign course of the disease; (f) the apparent responsiveness to sulphones in many cases. It is suggested that IgA pemphigus should be considered as an entity separate from, but closely related to, classic pemphigus.

Diagnosis, Differential↗
PubMed2006

Epidemiology of pemphigus in south-western Iran: a 10-year retrospective study (1991-2000).

BACKGROUND: Pemphigus is a disease demonstrating an uneven geographic distribution. This is the first epidemiologic study of pemphigus in Iran. METHODS: Data were collected from the files of all newly registered patients with pemphigus at the sole dermatologic referral center for south-western Iran located in the city of Shiraz, during the period 1991-2000. The data were analyzed with regard to age, sex, subtypes of pemphigus, clinical presentation, and season of onset. RESULTS: The results showed that the mean incidence of the disease for the overall population in Shiraz was 0.67/10(5)/year. The male to female ratio was 1 : 1.33. The mean age at onset of the disease was 38 years (range, 12-93 years). The disease was most common in the third to fifth decades (62.1% of the overall patients). Pemphigus vulgaris was the most commonly encountered type, followed by pemphigus foliaceus (87.7% and 9%, respectively). The first manifestations of the illness most often occurred during winter (30.8% of the patients). CONCLUSIONS: The results reveal a high incidence of pemphigus vulgaris in south-western Iran compared with that encountered in other countries. Our patients also had a relatively young age at onset and a female predominance.

Adolescent↗
PubMed2003

A unique form of endemic pemphigus in northern Colombia.

BACKGROUND: Endemic forms of pemphigus are a unique group of autoimmune diseases that represent opportunities to study interactions of the environment and genetics with the immune system. The restriction to relatively well-defined regions of South and Central America and perhaps Africa characterizes these diseases. OBJECTIVES: The aims of this study were to confirm the endemic nature of a new type of autoimmune disease occurring in a mining town in northeastern Colombia in the El Bagre area, to characterize it, and to compare it with other forms of endemic pemphigus. METHODS: A 10-year prospective, controlled epidemiologic, humanitarian, and immunologic fieldwork case-control survey was performed in El Bagre, Colombia. RESULTS: Our work revealed that this disease is endemic in rural areas surrounding El Bagre. The disease appeared in 4.7% of middle-aged and older men and postmenopausal women from these rural areas. This disease differs from previously described forms of endemic pemphigus. It shares some heterogeneous immunoreactivity with paraneoplastic pemphigus but is not associated with malignant tumors. The disease resembles Senear-Usher syndrome (pemphigus and lupus) but occurs endemically either with a localized stable clinical course or in a systemic form. This systemic form may affect organs other than skin and is characterized by episodic relapses and poor prognosis in comparison with the localized form. CONCLUSION: We have confirmed endemic pemphigus foliaceus in El Bagre as an autoimmune disease that shares features with Senear-Usher syndrome but occurs in an endemic fashion. Heterogeneous antigenic reactivity is observed as in paraneoplastic pemphigus but with no evidence of association with neoplasia. In addition, constant exogenous antigenic stimulation and a genetic predisposition may be required in the pathogenesis of this disease.

Autoimmune Diseases↗
PubMed1987

An autoantibody in pemphigus serum, specific for the 59 kD keratin, selectively binds the surface of keratinocytes: evidence for an extracellular keratin domain.

We have identified a novel IgG antikeratin autoantibody in the serum of a Brazilian pemphigus foliaceus patient (Cascas-42). This antibody is specific for the 59 kD acidic murine keratin and its 56.5 kD human counterpart (Moll's catalogue #10), and is distinct from the pemphigus antibody system. Antikeratin autoantibodies present in the Cascas-42 serum were purified by affinity chromatography with a 59 kD murine keratin-agarose column (IAP-Cascas-42 antibodies). The specificity of the IAP-Cascas-42 antibodies was tested by indirect immunofluorescence and immunoelectron microscopy against epidermal cryosections, trypsin-dissociated keratinocytes, and epidermal cell cultures. The serum was also tested with extracts from unlabeled and surface 125I-labeled keratinocytes (Iodo-Gen method) by immunoblot analysis of one- and two-dimensional polyacrylamide gel electrophoresis. The IAP-Cascas-42 antibodies bind the intercellular spaces of murine epidermis, and the cell surfaces of viable, dissociated murine keratinocytes, as well as murine epidermal cells in culture by immunofluorescence and immunoelectron microscopy. These autoantibodies did not stain cytoplasmic keratins and did not react with parallel human epidermal substrates. The Cascas-42 serum identified the 59 kD murine acidic keratin and its 56.5 kD human counterpart in epidermal extracts by two-dimensional polyacrylamide gel electrophoresis and immunoblot analysis. In addition, surface radioiodination of viable murine keratinocytes selectively labeled the 59 kD keratin suggesting that a domain of this molecule is exposed on the cell surface. The 125I-labeled 59 kD keratin was also recognized by the Cascas-42 serum by immunoblotting and autoradiography. These studies suggest that in murine epidermis, the 59 kD keratin is a transmembrane protein with an extracellular domain recognized by the IAP-Cascas-42 antibodies.

Animals↗
PubMed1994

Autoantibodies against cell adhesion molecules in pemphigus.

cDNA cloning has demonstrated that pemphigus autoantigens of both pemphigus vulgaris (PV) and pemphigus foliaceus are members of the desmoglein subfamily of the cadherin supergene family. The availability of these cDNAs allowed us to utilize molecular engineering to attempt to understand the pathophysiology of pemphigus. Transfection study with a chimeric molecule containing the extracellular domain of PV antigen (PVA) and the cytoplasmic domain of E-cadherin demonstrated that the extracellular domain of PVA mediates weak homophilic cell adhesion. Bacterial fusion proteins representing different parts of PVA showed that the major immunogenic domains are EC1, EC2, and EC4 and that at least one pathogenic epitope is located on the amino-terminal region of PVA, an area thought to be important for classic cadherin homophilic interaction. Further, a secreted form of PVA recombinant protein, PVIg, was produced by baculovirus expression. Immunoabsorption assay has demonstrated that PVIg is capable of absorbing pathogenic autoantibodies from patients' sera and preventing blister formation in neonatal mice.

Animals↗
PubMed2004

Evaluation of recombinant antigen-based assays for diagnosis of bullous autoimmune diseases.

The diagnosis of autoimmune bullous diseases is based on clinical observation and on the presence of autoantibodies directed to molecules involved in the adhesion systems of the skin. Immunofluorescence assays are the currently accepted method for detection of autoantibodies; such assays depend greatly on the skill of operators and are difficult to standardize. Recombinant desmoglein-1 (Dsg1), Dsg3, and BP180 peptides, the main autoantigens in pemphigus or bullous pemphigoid, have been used to develop new quantitative enzyme immunoassays (EIA) for the detection of specific antibodies. The present study was undertaken to evaluate the sensitivity and specificity of these immunoassays and to determine the correlation between the results and the clinical aspects of diseases. Serum samples from patients with pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, or mucous membrane pemphigoid, from healthy individuals, and from patients with unrelated autoimmune conditions were tested. Anti-desmoglein reactivity was detected in all the patients with pemphigus and in none of the controls. Patients with the more benign form of cutaneous disease had anti-Dsg1 antibodies, while patients with deeper cutaneous lesions or with mucosal involvement had anti-Dsg3 reactivity also, or exclusively. The BP180-based assay was positive for 66.6% of patients with bullous pemphigoid and for none of the patients with mucous membrane pemphigoid, and no reactivity was detected in the control sera. In conclusion, the anti-Dsg1 and anti-Dsg3 assays are useful in the diagnosis of pemphigus and provide information on the clinical phenotype of the disease. However, the sensitivity of EIA for detection of autoantibodies in bullous pemphigoid should be improved by the use of additional antigens or epitopes.

Antibody Specificity↗
PubMed2006

Non-pathogenic anti-desmoglein 3 IgG autoantibodies in Fogo Selvagem.

The endemic form of pemphigus foliaceus, fogo selvagem, is caused by IgG autoantibodies directed against desmoglein 1 (Dsg1). Hilario-Vargas and his colleagues describe a high prevalence of IgG autoantibodies against Dsg3, the target antigen of pemphigus vulgaris, in a Brazilian population where fogo selvagem is endemic, although those patients do not develop any apparent clinical phenotype of pemphigus vulgaris.

Autoantibodies↗
PubMed2002

A nonfatal case and 2 fatal cases of paraneoplastic pemphigus: can a complement indirect immunofluorescent test help to identify fatal "group A" paraneoplastic pemphigus cases?

We studied 3 recent cases of paraneoplastic pemphigus (PNP) in detail. Two patients died despite concerted management efforts. One patient received no treatment after the appearance of PNP and recovered completely from both PNP and lymphoma. Multiple serum studies of these 3 patients plus 9 other proven PNP cases revealed that 8 of 9 fatal PNP cases (referred to here as "group A") had distinctive cell surface antibodies detected by complement indirect immunofluorescent (CIIF) tests on monkey esophagus sections. By contrast, none of the sera from 3 patients with PNP who experienced long-term survival (referred to here as "group B") and none of 20 pemphigus vulgaris or 10 pemphigus foliaceus control sera revealed similar beaded cell surface CIIF reaction patterns, a difference that is statistically significant (P <.0001). Cell surface CIIF reaction patterns of group A PNP antibodies resemble the pattern of pemphigus antibody reactions in indirect immunofluorescent tests on the same substrate; however, the latter tend to be thinner and more linear, whereas the cell surface CIIF pattern tends to be more beaded, suggesting possible desmosomal reactions. We believe this test is useful in identifying an aggressive group A form of PNP.

Antibodies, Antinuclear↗
PubMed1986

Further analysis of pemphigus autoantibodies and their use in studies on the heterogeneity, structure, and function of desmosomes.

Pemphigus is an autoimmune disease that causes blistering of human epidermis. We have recently shown that autoantibodies in the serum of three pemphigus patients bind to desmosomes (Jones, J. C. R., J. Arnn, L. A. Staehelin, and R. D. Goldman, 1984, Proc. Natl. Acad. Sci. USA., 81:2781-2785), and we suggested that pemphigus blisters form, at least in part, from a specific antibody-induced disruption of desmosomes in the epidermis. In this paper, experiments are described that extend our initial observations. 13 pemphigus serum samples, which include four known pemphigus vulgaris (Pv) and four known pemphigus foliaceus (Pf) serum samples, have been analyzed by both immunofluorescence and by immunoblotting using cell-free desmosome preparations. Tissue sections of mouse skin processed for double indirect immunofluorescence using each of the pemphigus serum samples and a rabbit antiserum directed against a component of the desmosomal plaque (desmoplakin) show similar punctate cell surface staining patterns. This suggests that all 13 pemphigus serum samples contain autoantibodies that recognize desmosomes. These autoantibodies appear specific for stratified squamous epithelial cell desmosomes and do not recognize desmosomes of other tissues (e.g., mouse heart and mouse intestine). Cultured mouse keratinocytes, which possess well-defined desmosomes, were processed for indirect immunofluorescence using the pemphigus serum samples. Eight of the 13 sera (including the four known Pv samples but not the known Pf sera) stain desmosomes in these preparations. By double indirect immunofluorescence the desmoplakin antiserum stains a double fluorescent line along the contacting edges of cultured keratinocytes, whereas the positive pemphigus serum samples stain a single fluorescent line along this same border. We believe that these pemphigus autoantibodies recognize extracellular antigens located somewhere within the region between the two apposing membranes that comprise the desmosome. The pemphigus sera exhibit positive immunoblotting reactions with desmosome-enriched fractions obtained from bovine tongue epithelium. Three serum samples (including two of the four known Pf serum samples) react with 160- and 165-kD desmosome-associated polypeptides (Koulu, L., A. Kusimi, M. S. Steinberg, V. Klaus-Kovtun, and J. R. Stanley, 1984, J. Exp. Med., 160:1509-1518). Another eight serum samples (including the four known Pv sera) recognize a 140-kD desmosome-associated polypeptide. We propose that the antigens recognized by these human autoantibodies may play important roles in the adhesion of cells within the epidermis.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1991

Complement allotypes in Japanese patients with pemphigus and bullous pemphigoid.

The allotypes of C6, C7, factor B (BF) and factor I (IF) of the human complement system were studied in 11 Japanese patients with pemphigus (5 with pemphigus vulgaris and 6 with pemphigus foliaceus) and 17 with bullous pemphigoid (BP) to investigate the genetic background of these diseases. The allotypes were detected by using isoelectric focusing and immunoblotting. The frequency for IF*A allele in the pemphigus patients was significantly higher (p = 0.009) than that in healthy controls (n = 60). A significant association of IF A allotype with pemphigus was also observed (p = 0.027), with a relative risk of 6.3. There was no association between the C6, C7, BF or IF allotypes and BP. These data suggest that IF A allotype may be an etiological genetic factor in the development of pemphigus.

Alleles↗
PubMed1993

Erythema annulare-like acantholytic dermatosis (EAAD): nonbullous pemphigus or a new entity?

This article describes a case of unusual annular erythema-like dermatosis, with histological features of pemphigus foliaceus (subcorneal acantholysis) and IgG antibodies in circulation and bound in vivo to the keratinocyte surface. The reactivity of the antibodies, restricted to human squamous epithelium, was unique, differing from that of all known forms of pemphigus. This also was confirmed by immunoprecipitation. The problem is that these circulating antibodies could be missed if not determined on human substrate. It is to be established whether such cases present a new type of pemphigus or a unknown dermatosis with an autoimmune response of a pemphigus type.

Acantholysis↗
PubMed1993

The distribution of IgA pemphigus antigen in human skin and the role of IgA anti-cell surface antibodies in the induction of intraepidermal acantholysis.

BACKGROUND AND DESIGN: IgA pemphigus is an uncommon intraepidermal vesiculopustular disease that has clinical and histologic similarity to subcorneal pustular dermatosis and pemphigus foliaceus. All patients have IgA antibodies bound to the epidermal cell surface, and half of the patients have circulating IgA anti-cell surface antibodies detected by standard immunofluorescence testing. We studied the distribution of IgA pemphigus antigen in human skin and the pathogenetic role of circulating IgA antibodies in the induction of intraepidermal vesicle formation. We used skin specimens from numerous sites of two cadavers, as well as from neonatal foreskin, and serum specimens of two patients with IgA pemphigus. OBSERVATIONS: Organ culture and immunofluorescence studies revealed the following: (1) IgA pemphigus antibodies bound preferentially to the granular layer in the vast majority of skin sites that were tested. In one cadaver, binding was preferential to the spinous layer of plantar and buttock skin. No binding was observed in oral and esophageal mucosa. (2) Neither bound nor circulating IgA antibody was complement fixing. (3) One IgA pemphigus serum specimen that was negative by standard immunofluorescence had IgA antibodies that bound the epidermal cell surface after incubation under explant culture conditions. (4) Both IgA pemphigus serum specimens induced acantholysis in skin explant cultures. CONCLUSIONS: When antibodies from one IgA pemphigus serum specimen are used, the expression of IgA pemphigus antigen in human skin shows regional variability, interindividual variability, and variability in the microscopic distribution within the epidermal cell layers. IgA pemphigus antibodies play a role in the pathogenesis of IgA pemphigus. The skin explant culture is more sensitive than is standard immunofluorescence to detect circulating IgA antibodies.

Acantholysis↗
PubMed1995

Atypical pemphigus associated with monoclonal IgA gammopathy.

We describe a 60-year-old woman with atypical pemphigus and IgA-lambda monoclonal gammopathy. Histopathologic study of vesiculopustular lesions showed intraepidermal acantholytic and neutrophilic blisters. Direct immunofluorescence revealed intercellular IgG deposition with concurrent deposits of IgA and C3. Indirect immunofluorescence and immunoblotting studies revealed that the patient had circulating IgG anti-intercellular antibodies that recognized the 150 kd desmoglein (pemphigus foliaceus antigen) in bovine desmosome preparation. Immunoblot studies with human epidermal extract showed that the IgG of this patient exclusively reacted with the 140 kd protein (between the 150 kd human desmoglein and the 130 kd human pemphigus vulgaris antigen), the nature of which is currently unknown. The patient also had IgA anti-intercellular autoantibodies, which reacted with the desmoglein in the bovine desmosome sample but did not show any reactivity in human epidermal extract.

Antibodies, Anti-Idiotypic↗
PubMed2001

Pemphigus in South Africa.

BACKGROUND: Pemphigus is an autoimmune disease characterized by intraepidermal blistering. We describe the demography, prevalence, clinical features, response to treatment, and human leukocyte antigen (HLA) characteristics of pemphigus in Kwa-Zulu Natal, South Africa. METHODS: All patients with pemphigus were prospectively recruited over 12 years from January 1987 to December 1999. The demography, clinical features, histology, and immunofluorescence (IF) were recorded. In a subset of patients, HLA tests were performed. RESULTS: One hundred and twelve patients had pemphigus. Pemphigus foliaceus (PF) was the commonest variant seen (62 patients) and 80% of these patients were black. The mean age was 43 years (12-93 years) and the male to female ratio was 1 : 1.4. Fifty patients had pemphigus vulgaris (PV), of whom 82% were Indian. The mean age of presentation of PV was 48 years (21-82 years). The male to female ratio was 1 : 1.7. There was no mucosal involvement in PF. PV patients had painful oral lesions. The mortality rate was 14% in the total sample (six in PV and two in PF). HLA-B8 was positive in 41% of patients with PF (P < 0.001). CONCLUSIONS: PF occurs more commonly in black people, while most cases in Indians present with the PV subtype. Pemphigus patients present with severe and extensive disease, and PV patients share features in common with patients from their land of origin (India), suggesting a genetic link.

Adult↗
PubMed2006

Inhibition of Rho A activity causes pemphigus skin blistering.

The autoimmune blistering skin diseases pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are mainly caused by autoantibodies against desmosomal cadherins. In this study, we provide evidence that PV-immunoglobulin G (IgG) and PF-IgG induce skin blistering by interference with Rho A signaling. In vitro, pemphigus IgG caused typical hallmarks of pemphigus pathogenesis such as epidermal blistering in human skin, cell dissociation, and loss of desmoglein 1 (Dsg 1)-mediated binding probed by laser tweezers. These changes were accompanied by interference with Rho A activation and reduction of Rho A activity. Pemphigus IgG-triggered keratinocyte dissociation and Rho A inactivation were p38 mitogen-activated protein kinase dependent. Specific activation of Rho A by cytotoxic necrotizing factor-y abolished all pemphigus-triggered effects, including keratin retraction and release of Dsg 3 from the cytoskeleton. These data demonstrate that Rho A is involved in the regulation of desmosomal adhesion, at least in part by maintaining the cytoskeletal anchorage of desmosomal proteins. This may open the possibility of pemphigus treatment with the epidermal application of Rho A agonists.

Blister↗