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PubMed2003

The plant lectin wheat germ agglutinin inhibits the binding of pemphigus foliaceus autoantibodies to desmoglein 1 in a majority of patients and prevents pathomechanisms of pemphigus foliaceus in vitro and in vivo.

Pemphigus foliaceus (PF) is a life-threatening autoimmune blistering skin disease caused by pathogenic IgG autoantibodies against desmoglein 1 (dg1), a desmosomal cadherin-type adhesion glycoprotein. Using lectins and glycosidases, we have shown that dg1 displays an N-glycosylation pattern of the complex triantennary type. We have found that lectins and glycosidases interfere with N-bound sugar residues on the amino-terminal ectodomain of dg1 and completely abolish, in vitro, the antigenicity of dg1 in most of the patients' sera. Moreover, in an ex vivo model using punch biopsies from normal human skin, we demonstrate that preincubation of the epidermis in wheat germ agglutinin (WGA) prevents PF autoantibody binding, acantholysis, and subcorneal blistering. In addition, we show that topical treatment with WGA inhibits PF autoantibody binding to keratinocytes in both newborn BALB/c mice and in organotypic human epidermis grafted onto the back of SCID mice. The epidermis of these pretreated animals displays a regular morphology, whereas control animals develop the immunopathologic phenotype of PF. These findings suggest that WGA may interfere with autoantibody binding to dg1, preventing experimental PF without affecting the adhesive function of dg1. Our observations may provide a new approach to the therapy of PF.

Administration, Topical↗

PubMed2003

High levels of interleukin-1 in patients with endemic pemphigus foliaceus.

Endemic pemphigus foliaceus (EPF) is an autoimmune disease characterized by blister formation with a loss of cohesion and infiltration of inflammatory cells. We observed that supernatants of peripheral blood mononuclear cells from patients produced significantly more interleukin-1beta (IL-1beta) than those from stimulated healthy controls. Furthermore, a Th2 bias was observed in EPF patients when the IL-5/gamma interferon ratio was analyzed. These results indicate that cells from pemphigus patients react with a vigorous proinflammatory response.

Autoimmune Diseases↗

PubMed1989

Complement activation in Brazilian pemphigus foliaceus.

Brazilian pemphigus foliaceus is an autoimmune blistering skin disease of man that has a very high incidence in a confined geographical distribution. Rocket immunoelectrophoresis of plasma showed increased levels of complement fragments, C4d and Ba, indicating activation of complement through both the classical and the alternative pathways. Less sensitive methods such as CH50, total C3 and C4 did not demonstrate this activation, and immune complex measurements were within normal range. While complement may not be absolutely necessary for the development of skin lesions, our longitudinal studies show that activation of complement is at its highest during the most active phase of the disease.

Acute Disease↗

PubMed1987

Human autoantibodies against a desmosomal protein complex with a calcium-sensitive epitope are characteristic of pemphigus foliaceus patients.

Pemphigus foliaceus (PF) patients have antibodies against a tightly, but noncovalently bound complex of polypeptides, which consists of desmoglein I (DGI) and other, possibly desmosomal, proteins. Most PF antibodies bind a calcium-sensitive epitope on this complex and chelation of calcium destroys the reactivity of these sera with the complex, but not the complex itself. The PF sera that do bind the desmosomal complex in the absence of calcium are those sera capable of binding denatured DGI on immunoblotting, and these same sera also immunoprecipitate only DGI when the desmosomal complex is dissociated with SDS. These findings demonstrate that autoantibodies against a complex of desmosome-associated proteins are characteristic of PF and define a calcium-sensitive conformational epitope on this complex.

Animals↗

PubMedDate not supplied

[Tests of inflammatory activity in endemic pemphigus foliaceus].

Endemic pemphigus foliaceus (EPF) has its pathogenesis frequently associated to autoimmune phenomena. In this paper, a few routine laboratory tests, usually disturbed in some autoimmune diseases, were taken in 20 patients with EPF, which were screened for antinuclear antibodies (ANA), rheumatoid factor (RF), C-reactive protein (CRP) and changes of erythrocyte sedimentation rate (ESR), serologic proteins electrophoresis and total leucocyte count. The CRP was found in 60% of cases, leukocytosis in 85%, high ESR in all of them and mild alterations in serologic proteins analysis. No ANA or RF was found. Although widely accepted as nonspecific tests, we believe that an association of the laboratory routine tests with clinical findings, can prove to be helpful in the follow up care of these patients.

Acute Disease↗

PubMed2004

Dermal dendritic cell number correlates with serum autoantibody titers in Brazilian pemphigus foliaceus patients.

Pemphigus foliaceus (PF) is an autoimmune bullous disease endemic in Brazil. Since serum IL-12 is increased in patients with PF and Langerhans cells (LC) produce IL-12, we titrated serum autoantibodies by indirect immunofluorescence, and quantified epidermal dendritic cells, known as LC, and dermal dendritic cells (DC). Biopsies of blistering lesions were obtained from 22 patients, 13 of whom were submitted to biopsy of both injured and of apparently healthy skin. The control groups consisted of skin from 8 cadavers and from 12 women submitted to breast plastic surgery. LC and DC were identified with anti-CD1a antibody and quantified by morphometric analysis. LC number in the lesion and in apparently healthy skin from PF patients was similar to that of both control groups. DC number in the injured skin (median=0.94 DC/mm basement membrane) was higher than that of the cadaver group (median=0.13 DC/mm basement membrane). In the 13 patients with biopsies of both injured and apparently healthy skin, LC and DC were present in larger numbers in the lesion. There was a direct correlation between DC number in the lesion of the PF group and serum autoantibody titers. This correlation was not observed for LC number. The increased number of DC in the lesion, as well as its direct correlation with serum autoantibody titers suggest the participation of DC in the pathogenesis of PF. The relationship between increased DC number and IL-12 in PF needs to be clarified.

Autoantibodies↗

PubMed2003

The role of subclass switching in the pathogenesis of endemic pemphigus foliaceus.

Endemic pemphigus foliaceus, like the sporadic form seen in the developed world, is mediated by IgG antibodies to desmoglein-1. We studied an endemic focus in Limao Verde, Brazil, where disease prevalence is 3.4%. We previously detected IgG antibodies to desmoglein-1 in 97% of patients, but also in 55% of normal subjects in the endemic focus, with progressively lower levels in normal subjects in surrounding areas. An environmental trigger is hypothesized to explain these and other findings. In this study we sought to determine if patients and enzyme-linked-immunosorbent-assay-positive normal subjects in Limao Verde differ in IgG subclass response to desmoglein-1. We developed a sensitive and specific subclass enzyme-linked immunosorbent assay using recombinant desmoglein-1 and standardized the assay to enable comparability between the four subclasses. We found that normal subjects have an IgG1 and IgG4 response, whereas patients have similar levels of IgG1 but a mean 19.3-fold higher IgG4 response. Patients in remission have a weak IgG4 response, and a 74.3-fold higher IgG4 response is associated with active disease. Finally, in five patients in whom we had blood samples from both before and after the onset of clinical disease, a mean 103.08-fold rise in IgG4 was associated with onset of clinical disease, but only a mean 3.45-fold rise in IgG1. These results suggest that the early antibody response in normal subjects living in the endemic area and in patients before the onset of clinical disease is mainly IgG1. Acquisition of an IgG4 response is a key step in the development of clinical disease.

Adolescent↗

PubMed1984

Distinction between epidermal antigens binding pemphigus vulgaris and pemphigus foliaceus autoantibodies.

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases in which antibodies develop to the cell surface of epidermal cells. In this study we sought to determine the antigenic specificity of antibodies in the sera of patients with PV and PF. Sera from 12 patients with PV were used to immunoprecipitate extracts of cultured human epidermal cells that were radiolabeled with 14C-amino acids. Immunoprecipitates were identified by SDS polyacrylamide gel electrophoresis (PAGE) and fluorography. All 12 PV sera precipitated a protein which, when reduced, displayed chains of 130,000 and 80,000 mol wt on SDS-PAGE. Electrophoresis under nonreducing conditions identified a 210,000-mol wt molecule, which was presumably formed by disulfide crosslinking of the 130,000 and 80,000-mol wt chains. Immunoprecipitates of epidermal cell extracts that were labeled with 14C-glucosamine indicated that the 130,000-mol wt chain. Seven of eight PF sera, which were run concurrently with the PV sera in this immunoprecipitation assay, did not precipitate this glycoprotein, nor did they specifically precipitate any protein. To determine if a specific molecule which reacted with antibodies in PF sera could be identified, we used immunoblot analysis of extracts of normal human epidermis. The proteins in these extracts were reduced, separated by SDS-PAGE, and electrophoretically transferred to nitrocellulose sheets or to 2-aminophenylthioether paper. Immunoperoxidase staining of the transferred proteins with PF sera indicated that four of eight PF sera contained antibodies that stained a protein band of 160,000 mol wt. Indirect immunofluorescence, using normal human skin as the substrate, indicated that IgG that was eluted from this protein band stained the epidermis in a cell surface pattern. PV sera did not specifically recognize any bands by immunoblot analysis. Immunoblots performed with PV antigen that was immunoprecipitated from cell culture extracts suggested that, once denatured for SDS-PAGE, PV antigen is no longer immunoreactive. Taken together, these data indicate that: autoantibodies contained in PV sera from various patients have a unique molecular specificity; autoantibodies from most PF sera have a specificity different from that of PV autoantibodies; and autoantibodies from various PF patients may not have identical antigenic specificities. These differences in antigenic specificity between PV and PF sera may account for the clinical and histologic differences between these diseases.

Antigen-Antibody Complex↗

PubMed1978

Absence of intercellular antigens in the deep layers of the epidermis in pemphigus foliaceus.

12 patients with pemphigus foliaceus, a form of pemphigus with lesions that arise in the intercellular substance in the superficial layers of the epidermis, and 7 patients with pemphigus vulgaris, where lesions are in the deep layers, were studied by immunofluorescence. Circulating antibodies to intercellular antigens (IC antibodies) were found in 11 pemphigus foliaceus and 5 pemphigus vulgaris patients. On direct immunofluorescence of skin lesions 75% (9 of 12), pemphigus foliaceus patients had intercellular deposits of IgG localized solely or predominantly in the superficial epidermal layers, whereas this was not the case in any of the patients with pemphigus vulgaris. Over 70% of the pemphigus foliaceus patients with predominantly superficial IgG deposits lacked in their lesions normal intercellular antigens usually expressed in the deep layers of the epidermis. This was shown by the inability of IC antibodies in autologous or allogeneic sera to bind to intercellular antigens in the lower epidermis of patient's skin, even though the same sera could bind to intercellular antigens in all layers of normal allogeneic skin. Lack of normal intercellular antigens deep in the epidermis may result in circulating IC antibodies binding to the superficial layers, a site which corresponds to, and thus in some patients may account for, the anatomical location of lesions in pemphigus foliaceus.

Antibody Specificity↗

PubMed1996

[Comparative epidemiology of pemphigus in Tunisia and France. Incidence of foliaceus pemphigus in young Tunisian women].

INTRODUCTION: Recent studies have suggested that pemphigus foliaceus is quite frequent in young Tunisian women. In order to confirm this hypothesis, we compared the incidence of pemphigus in general in Tunisia with that in the Ile-de-France region. METHOD: All new cases of pemphigus diagnosed during a 6-year period were reviewed in our dermatology and pathology laboratories. These cases were classed as pemphigus foliaceus or pemphigus vulgaris on the basis of histology reports. RESULTS: In France, the incidence was 1-7 new cases per million per year (95 p. 100 confidence interval 1.4-2.1). Pemphigus vulgaris was diagnosed in 73 p. 100 of the cases with an incidence increasing with age. Sex ratio (F/M) was 1.2. The incidence observed in Tunisia was significantly higher than that observed in France with 6.7 new cases per million per year (95 p. 100 confidence interval 5.8-7.7). Pemphigus foliaceus was more frequent (61 p. 100), the sex ratio (F/M) was 4.1. Incidence was higher in young women, with 20 new cases of pemphigus foliaceus per million per year among women from 25 to 34 years of age. These levels were higher in rural desert areas. No familial cases were observed and only one case occurred in a child. DISCUSSION: These findings confirm the specific epidemiology of pemphigus in Tunisia, which appears to be similar and also different from that in Brazilian pemphigus. As in Brazil, there was a predominance of pemphigus foliaceus in young adults living in rural areas in poor socioeconomic conditions. However in Tunisia the disease predominates significantly in women and there are no familial and rare juvenile cases.

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↗

PubMed1986

Antigenic specificity of fogo selvagem autoantibodies is similar to North American pemphigus foliaceus and distinct from pemphigus vulgaris autoantibodies.

Fogo selvagem (FS) is clinically, histologically, and immunopathologically similar to sporadic pemphigus foliaceus (PF, as seen in North America and Europe), although the epidemiology of these 2 diseases differs markedly. It has been proposed that FS is identical to PF but, for some reason, occurs in an endemic focus in central Brazil. If this hypothesis is correct, the autoantibodies in FS and PF should have similar antigenic specificities. We studied sera from 13 patients with FS from central Brazil, and compared them with 20 sera from patients with PF from the United States. All these sera had circulating antibodies that bound the cell surface of epithelial cells in identical patterns by indirect immunofluorescence on monkey esophagus or normal human skin. In immunoprecipitation studies none of the 13 FS sera precipitated the pemphigus vulgaris (PV) antigen from radiolabeled extracts of cultured human keratinocytes. This is similar to the findings with PF sera of which 19 of 20 sera did not react with PV antigen, but in sharp contrast to the results with PV sera which, as previously reported, all immunoprecipitate the PV antigen. Immunoblotting performed on extracts of normal human epidermis demonstrated that 7 of 20 PF sera specifically and intensely bound an approximately 160 kD polypeptide, previously identified as desmoglein I, a desmosomal glycoprotein. Similarly, 3 of 13 FS sera specifically bound a 160 kD polypeptide. Thirteen normal sera from North America, 8 normal and disease control sera from central Brazil, 11 PV sera, and 12 bullous pemphigoid sera did not specifically bind this polypeptide. Two-dimensional gel electrophoresis confirmed that the 160 kD polypeptides identified by the subgroup of PF and FS sera were identical. These studies demonstrate that, although the exact molecular specificities of the majority of PF and FS sera remain to be determined, FS autoantibodies do not bind the PV antigen and a subgroup of FS autoantibodies have molecular specificity identical to that of a subgroup of PF autoantibodies.

Antibody Specificity↗

PubMed2000

Transplacental passage of maternal pemphigus foliaceus autoantibodies induces neonatal pemphigus.

The association of maternal pemphigus foliaceus (PF) with neonatal PF is rare and may be secondary to transplacental passage of PF autoantibodies. We describe a 25-year-old patient with PF who was delivered of two consecutive babies, one with classic skin lesions of PF and another that was normal. The neonate with PF was born when the mother had widespread skin disease; the normal newborn was born when the mother was in partial remission. The titers of PF autoantibodies were higher in the mother's serum and the cord serum of the baby with PF than in the mother during partial remission and the unaffected baby. The mother and affected baby had autoantibodies to desmoglein 1. Furthermore, cord blood from the baby with PF induced skin disease when injected into mice. In this case, maternal PF was associated with neonatal PF when the titers of maternal anti-desmoglein 1 autoantibodies were elevated. The cutaneous disease in neonatal PF is due to anti-desmoglein 1 autoantibodies.

Adult↗

PubMed2000

HLA class II polymorphism contributes to specify desmoglein derived peptides in pemphigus vulgaris and pemphigus foliaceus.

Susceptibility to Pemphigus, an autoimmune disease of the skin, has been previously linked to DRB1*0402, 1401/04 and DQB1*0503 in pemphigus vulgaris (PV), to DRB1*0102, 0404, 1402/06 in endemic pemphigus foliaceus in Brazil and to DRB1*04 in Italian patients suffering from pemphigus foliaceus (PF). The disease is caused by autoantibodies against desmoglein (Dsg1 in PF, Dsg3 in PV). Molecular typing of 57 French patients suffering from PV (37) and from PF (20) confirmed previous results concerning PV and showed that DRB1*0102 and 0404 are susceptible molecules to PF in France. We have analysed the characteristics of the 'pockets' of the susceptibility-associated molecules to PV and PF and we showed that (i) in PV, two kinds of Dsg3 derived peptides may be presented by HLA-DR according to HLA polymorphism (DRB1*0402 or DRB1*14/0406), (ii) the same Dsg1 peptides may be presented by DRB1*0102, DQB1*0404 or DRB1*14 in PF, (iii) the DRB1*14/0406 PV-related molecules may be able to present Dsg1 and Dsg3 peptides thereby providing an explanation for the cases of PV with combined responses to Dsg1 and to Dsg3 which are typified by a muco-cutaneous clinical phenotype.

Alleles↗

PubMed2003

Neonatal pemphigus foliaceus.

The term "pemphigus" refers to a group of diseases that are characterized by the presence of cutaneous or mucosal blisters and erosions, and antiepidermal autoantibodies. There are several case reports of neonatal pemphigus vulgaris in the literature. Although pemphigus foliaceus antibodies have been shown to cross the placenta, to our knowledge, this is only the second reported case of neonatal pemphigus foliaceus. The proposed mechanism of disease transfer is the passive transfer of maternal IgG antibodies across the placenta.

Administration, Topical↗

PubMed2002

Nonendemic pemphigus foliaceus in children.

BACKGROUND: Pemphigus foliaceus is a cutaneous, autoimmune, blistering disease comprising two major categories: endemic and sporadic. The endemic form, also known as fogo selvagem, primarily affects children and young adults in rural Brazil. In contrast, the sporadic form of pemphigus foliaceus is generally a disease of the middle-aged and elderly. OBJECTIVE AND METHODS: Because the sporadic form of pemphigus foliaceus rarely affects children, information specific to this unique group is lacking. We describe a 3-year-old boy with the disease and retrospectively review data from 28 past cases. RESULTS: In comparison to pediatric cases of pemphigus vulgaris, sporadic pemphigus foliaceus in children tends to follow a generally benign course of relatively short duration. However, long-term outcome studies are lacking. A pattern of skin lesions described as "arcuate," "circinate," or "polycyclic" appears to be a unique and specific presentation of this disease in children. Occasionally, as in our case, the diagnosis may prove difficult to establish by using routine histology or immunopathology. CONCLUSION: The commercial availability of antigen-specific techniques such as enzyme-linked immunosorbent assay for serum desmoglein 1 autoantibody should eliminate delay in diagnosis. Hydroxychloroquine may be another treatment option for those children with photodistributed lesions. Further experience and long-term outcome studies in children are needed to determine whether some medication side effects may outweigh the risks from the disease itself.

Anti-Inflammatory Agents↗

PubMed1993

Desmocollins I and II are recognized by certain sera from patients with various types of pemphigus, particularly Brazilian pemphigus foliaceus.

Recently, it has been shown that desmoglein, pemphigus foliaceus target antigen, and a 130-kD pemphigus vulgaris antigen belong to the cadherin family of cell adhesion molecules. We tried to determine whether desmocollins I/II, other cadherin-like transmembranous glycoproteins present in desmosomes, are also recognized by pemphigus autoantibodies of the IgG class. We examined 16 pemphigus vulgaris sera, 15 pemphigus foliaceus sera, 15 Brazilian pemphigus foliaceus sera, five bullous pemphigoid sera, and 65 normal sera. Four (25%) pemphigus vulgaris sera, one (7%) pemphigus foliaceus serum, eight (53%) Brazilian pemphigus foliaceus sera, and three (5%) normal sera reacted with desmocollins I/II on immunoblots of bovine desmosome preparation. The affinity-purified desmocollins I/II pemphigus autoantibodies were shown to bind the epidermal cell surface by indirect immunofluorescence. Immunoblot analysis revealed one pemphigus vulgaris serum, one Brazilian pemphigus foliaceus serum, and one normal serum recognizing a recombinant protein produced by a desmocollin cDNA clone. Moreover, immunoblot analysis of reactivity of a Brazilian pemphigus foliaceus serum with recombinant proteins produced by deletion mutants of the desmocollin cDNA clone showed that the extracellular portion of desmocollin is immunogenic in this pemphigus patient. We conclude that desmocollins I/II are recognized by certain sera from patients with various types of pemphigus, particularly Brazilian pemphigus foliaceus. However, the significance of this reactivity remains to be defined.

Animals↗

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↗