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PubMed2006

Antibodies against desmoglein 1 in healthy subjects in endemic and nonendemic areas of pemphigus foliaceus (fogo selvagem) in Peru.

BACKGROUND: Endemic pemphigus foliaceus or fogo selvagem is an autoimmune skin disease characterized by the presence of subcorneal superficial blisters and antibodies of the immunoglobulin G4 (IgG4) class specific for the desmosomal glycoprotein, desmoglein 1. In Peru, no studies have been published on the seroprevalence of antibodies against desmoglein 1 in healthy subjects from endemic foci. SUBJECTS AND METHODS: This was a cross-sectional study. The sample included 82 healthy subjects, 41 from the Pueblo Libre community, a focus of endemic pemphigus foliaceus, and 41 from a nonendemic urban area in Pucallpa City. Enzyme-linked immunosorbent assay (ELISA) was used to determine the presence of antibodies against desmoglein 1. Samples were processed and tested at the Department of Dermatology and Cutaneous Surgery, School of Medicine, University of Miami, Miami, Florida. RESULTS: It was found that 31.7% of healthy individuals (13 subjects) from the endemic focus had anti-desmoglein 1 antibodies. A statistically significant association was found between the distance from the endemic focus and the presence of antibodies against desmoglein 1 in subjects living within the endemic focus [Mantel-Haenszel odds ratio (OR), 3.34; P = 0.03; 95% confidence interval (CI), 1.06-10.48]. Agriculture as an occupation showed a statistically significant association with the presence of antibodies against desmoglein 1 (Mantel-Haenszel OR, 7.84; P < 0.001; 95% CI, 2.47-24.87). CONCLUSIONS: Antibodies against desmoglein 1 are present in healthy subjects exposed to an endemic focus of pemphigus foliaceus (fogo selvagem). Agriculture is associated with a high risk of development of antibodies against desmoglein 1 in the endemic focus of the Pueblo Libre community.

Adolescent↗
PubMed1996

Curvicircular intracytoplasmic membranous structures in keratinocytes of pemphigus foliaceus.

We noticed intracytoplasmic membranous, annular, or circular structures in the lesion of pemphigus foliaceus and studied these by regular transmission electron microscopy and immunoelectron microscopy. These curvicircular bodies were observed in the preacantholytic keratinocytes of the blister wall as well as in acantholytic cells in 6 out of 6 patients with pemphigus foliaceus. They were absent in samples from 3 patients with pemphigus vulgaris. These structures were about 60-70 nm wide and consisted of 4 electron-dense layers. They were continuous with intact desmosomal structures and gap junctions in the periphery of the keratinocytes. These curvicircular membranous bodies were well labeled with immunogold particles for desmoglein, plakoglobin, connexin 43, and IgG. In contrast to pemphigus vulgaris, splitting of desmosomes through dissolution of intercellular desmoglea was seldom observed in all 6 specimens of pemphigus foliaceus. These findings suggest that in pemphigus foliaceus 1) curvicircular bodies are derived from internalized desmosomes and gap junctions, and 2) cell-to-cell adhesions are weakened by this internalization and acantholysis is initiated, while in pemphigus vulgaris the dissolution of desmoglea is the initial event. It is suggested that in pemphigus foliaceus the binding of autoantibody induces internalization of many intact desmosomes and gap junctions rather than splitting them.

Fluorescent Antibody Technique↗
PubMed1998

Lack of mucosal involvement in pemphigus foliaceus may be due to low expression of desmoglein 1.

Oral mucosal lesions are seen in most cases of pemphigus vulgaris, whereas they are only rarely seen in pemphigus foliaceus; however, both pemphigus vulgaris and pemphigus foliaceus sera show positive immunofluorescence staining on oral mucosa. To explain this apparent paradox, we examined the expression level of desmoglein (Dsg)3, pemphigus vulgaris antigen, and Dsg1, pemphigus foliaceus antigen, in human squamous mucosal epithelia and epidermis by immunofluorescence staining and immunoblotting. For immunofluorescence staining, Dsg isotype-specific antibodies were produced by immunoadsorbing pemphigus vulgaris sera with either recombinant Dsg1 or Dsg3 baculoprotein. In oral mucosa and esophagus both Dsg were immunoreactive on cell surfaces throughout the entire epithelia, but staining intensity was weaker for Dsg1 than for Dsg3. Immunoblotting was performed to compare Dsg1 and Dsg3 expression levels in extracts from epidermis and oral mucosa. The total amount of desmosomal proteins applied was adjusted to give the same degree of staining intensity for desmoplakin, a cytoplasmic plaque protein of desmosomes. In the mucosal extract, the Dsg1 band was much weaker than Dsg3, whereas in the epidermal extract the Dsg1 band was stronger than Dsg3. These data suggest that although Dsg1 and Dsg3 are expressed in a similar distribution throughout squamous mucosal epithelia, Dsg1 is expressed at a much lower level than Dsg3. This finding provides a good explanation for the paradox: even though anti-Dsg1 autoantibodies block the function of Dsg1 in the mucosal epithelia, Dsg3 may be sufficient for cell-cell adhesion, with consequently no apparent oral involvement in pemphigus foliaceus patients.

Autoantigens↗
PubMed1990

A soluble and immunoreactive fragment of pemphigus foliaceus antigen released by trypsinization of viable human epidermis.

Pemphigus foliaceus (PF) antigen is a transmembrane desmosomal glycoprotein (desmoglein I), part of which is located on the keratinocyte surface. Previous studies have shown that after trypsinization of viable human epidermis, this antigen is no longer detected on the surface of detached keratinocytes. It was not known, however, if this loss of antigenic activity was due to destruction, internalization, or cleavage of the antigen itself. In the present study we investigated the fate of the PF antigen after trypsinization of viable human skin. By using Concanavalin-A agarose affinity chromatography, we could partially purify an antigenic glycoprotein fraction that was released by trypsinization into the medium. This antigenic fraction was radiolabeled and tested by immunoprecipitation using sera from endemic pemphigus foliaceus or fogo selvagem (FS), non-endemic pemphigus foliaceus (NEPF), pemphigus vulgaris (PV), and bullous pemphigoid (BP) patients, and sera from normal subjects as controls. Immunoprecipitated labeled proteins were analyzed by SDS-PAGE and autoradiography. All FS sera (20 of 20 FS and five of five NEPF) and 46% of the PV sera (six of 13) immunoprecipitated a band of 45-kD molecular weight. Sera from FS patients in prolonged clinical and serological remission (seven of 10), sera from BP patients (five of five), and sera from normal donors (nine of nine) did not precipitate this 45-kD band. This study showed that a fragment of the PF antigen is released by trypsinization of human skin as a soluble immunoreactive glycopeptide of 45-kD molecular weight. Additionally, this procedure has generated sufficient quantities of the PF antigen for further biochemical characterization.

Antigens, Surface↗
PubMed2000

UVB-induced acantholysis in endemic Pemphigus foliaceus (Fogo selvagem) and Pemphigus vulgaris.

BACKGROUND: The autoantibody-mediated disease in fogo selvagem (FS) is aggravated by sunlight exposure. OBJECTIVE: The aim of this study was to test whether UVB exposure of uninvolved skin of patients with FS (n = 21) enhances epidermal acantholysis and in vivo binding of FS autoantibodies as compared with a control group (n = 4). Another control group included 8 patients with pemphigus vulgaris (PV). RESULTS: Exposure of uninvolved skin of FS to UVB induces acantholysis (15/21 patients) with in vivo binding of IgG and C3 to the epidermal intercellular spaces (17/21 patients). Similar results were found in PV (6/8 patients). The test was negative in the control group. CONCLUSION: Sunlight exposure is harmful in the clinical course of patients with FS and those with PV. Protection from sunlight should be part of the treatment of these patients.

Acantholysis↗
PubMed2002

A subset of pemphigus foliaceus patients exhibits pathogenic autoantibodies against both desmoglein-1 and desmoglein-3.

In pemphigus vulgaris the major pathogenic antibody binds desmoglein-3, and mediates mucosal disease. Development of cutaneous disease is associated with acquisition of antibodies to desmoglein-1. In pemphigus foliaceus, and its endemic form, fogo selvagem by contrast, the major pathogenic antibody recognizes desmoglein-1 and mediates cutaneous disease only. In this study, we sought to determine the prevalence of antibodies to desmoglein-3 in patients with pemphigus foliaceus and fogo selvagem. We produced recombinant desmoglein-1 and desmoglein-3, and used them in highly sensitive and specific enzyme-linked immunosorbent assays, as well as immunoprecipitation assays. We detected antibodies to desmoglein-3 in 19 of 276 patients with pemphigus foliaceus and fogo selvagem, who had cutaneous disease only. We showed that these antibodies to desmoglein-3 could be absorbed in a concentration-dependent manner by desmoglein-3 but not by desmoglein-1. Also antibodies to desmoglein-1 could be absorbed in a concentration-dependent manner by desmoglein-1 but not desmoglein-3. This suggests that two separate species of antibody are present rather than one antibody capable of cross-reacting with both desmoglein-1 and desmoglein-3. Finally, it was shown that affinity-purified antibodies to desmoglein-3 from patients with pemphigus foliaceus and fogo selvagem induced a pemphigus vulgaris-like skin disease in mice by passive transfer. These results suggest that a subset of patients with pemphigus foliaceus and fogo selvagem have antibodies to desmoglein-3 that may be involved in the pathogenesis of their cutaneous disease.

Animals↗
PubMed2002

Altered immunohistochemical staining for desmoglein in skin biopsies in canine pemphigus foliaceus.

In this study, 50 cases of canine pemphigus foliaceus and 49 cases of canine superficial pyoderma were examined by immunohistochemical staining for patterns of desmoglein expression. In 31/50 (62%) of pemphigus foliaceus cases, there was an altered staining pattern for desmoglein consisting of distinct clumped deposits at the periphery of keratinocytes and/or dark cytoplasmic staining of acantholytic cells (consistent with internalization of desmoglein). In contrast, desmoglein staining in biopsies from cases of superficial pyoderma was diffusely pale without evidence for clumping or distinct internalization. This study demonstrates that epidermal desmoglein expression is altered in some cases of pemphigus foliaceus in dogs and suggests that immunohistochemical staining for this protein may be useful in diagnosis.

Animals↗
PubMed1996

Effect of substrate on indirect immunofluorescence test for canine pemphigus foliaceus.

The effect of substrate on indirect immunofluorescence (IIF) tests for the detection of circulating autoantibodies was studied by examining sera from 14 canine pemphigus foliaceus patients, six sera with non-pemphigus dermatoses and ten normal dog sera against five different substrates from three species. These substrates included bovine esophagus, bovine nose, bovine tongue, monkey esophagus, and canine nose skin. Nine out of 14 (64.3%) sera from patients with canine pemphigus foliaceus showed intercellular space staining by indirect immunofluorescence using bovine esophagus as substrate. However, sera from nonpemphigus dermatoses and normal dog did not react with bovine esophagus. In other substrates, only bovine tongue showed 1/8 (12.5%) positive reaction at the intercellular space by sera from canine pemphigus foliaceus. Dog nose skin showed the intercellular space staining against ten of ten (100%) normal dog serum. Monkey esophagus showed the fluorescent deposit at the intercellular space in four of nine (44.4%) of pemphigus foliacues dog sera, however, four of ten (40%) of normal dog sera revealed nonspecificity intercellular staining. These results indicate that the sensitivity and the specifity of IIF test in canine pemphigus foliaceus depend on the substrate. The best substrate for detecting circulating autoantibody in canine pemphigus foliaceus patients among five different substrates was bovine esophagus because of its sensitivity and high specificity. The diagnosis of canine pemphigus foliaceus should be made on the basis of a combination of clinical signs, histopathology, direct immunofluorescence, and the detection of circulating autoantibody.

Animals↗
PubMed1984

Pemphigus foliaceus induced by rifampicin.

Pemphigus foliaceus was seen in a patient with pulmonary tuberculosis during rifampicin therapy. The skin lesions were eczematoid patches, flaccid bullae and crusted plaques on the trunk and extremities, histologically showing a high epidermal blister with acantholysis. The diagnosis was confirmed by immunofluorescent studies. After the patient discontinued rifampicin, the skin lesions cleared completely within 5 weeks without any systemic medication.

Humans↗
PubMed1989

The pathogenic effect of IgG4 autoantibodies in endemic pemphigus foliaceus (fogo selvagem).

Endemic pemphigus foliaceus, or fogo selvagem, is an autoimmune blistering skin disease caused by IgG autoantibodies to a desmosome-associated glycoprotein. We studied the IgG subclasses with autoantibody activity in serum from 29 patients with active disease and in the skin lesions of 18 patients by immunofluorescence, using IgG-subclass-specific monoclonal antibodies. The predominant disease autoantibodies present in all patients were of the IgG4 subclass. IgG1 and IgG2 autoantibodies were detected in low titer in the 29 patients: IgG1 in 23 patients and IgG2 in 9. IgG3 autoantibodies were not detected in the serum of any patient. Direct immunofluorescence testing of skin lesions showed a preferential deposition of IgG4 on the keratinocyte surface. The pathogenic effect of IgG4 was demonstrated by the passive transfer of fractions containing IgG4 autoantibodies from the patients to neonatal BALB/c mice. The disease of the patients was reproduced clinically, histologically, and immunologically in these animals. Only IgG4 autoantibodies were detected by direct immunofluorescence, bound to the epidermis in the lesions of the mice, and by immunoelectron microscopy at the keratinocyte surface. IgG4 has previously been reported to be a blocking or protective antibody because it has poor effector functions in vitro, as compared with the other IgG subclasses. The finding that it is the pathogenic autoantibody in fogo selvagem raises the possibility that it may also be important in other autoimmune disease.

Animals↗
PubMed2005

Ex vivo analysis of desmoglein 1-responsive T-helper (Th) 1 and Th2 cells in patients with pemphigus foliaceus and healthy individuals.

Pemphigus foliaceus (PF) is a severe autoimmune bullous disorder, characterized by autoantibodies (autoAb) against desmoglein 1 (Dsg1). As T cells may be critical in the pathology of PF, the aim of the present study was to identify and characterize autoaggressive T-helper cells reactive to Dsg1 in PF patients and healthy individuals. Eight patients with the clinical diagnosis of PF and six HLA class II-matched healthy individuals were examined. By magnetic cell-sorting (MACS) cytokine-secretion assay, Dsg1-responsive T-helper (Th) 1 and Th2 cells were isolated and cloned by limiting dilution. The generated T-cell clones (TCC) were characterized regarding proliferative response, TCR Vbeta-chain usage, and cytokine profile upon in vitro stimulation with Dsg1. Both Dsg1-reactive Th1 and Th2 cells were detected in PF patients and controls at similar frequencies. A total of 15 Th1 and Th2 clones were isolated from patients and 27 TCC from healthy controls. Analysis of TCR Vbeta-chain usage of autoreactive T cells from both groups revealed no predominance of a specific Vbeta chain. Noteworthy, the isolated TCC showed a polarized Th1- or Th2-like phenotype upon in vitro culture and stable expression of Th1 or Th2 cytokines during long-term in vitro culture. In summary, our data demonstrate that T-cell autoreactivity against Dsg1 is not restricted to patients with PF. Moreover, both Th1 and Th2 cells were present in patients and healthy donors, suggesting that the loss of B-cell tolerance against Dsg1 in PF is not exclusively determined by the presence of autoaggressive T cells.

B-Lymphocytes↗
PubMed2005

Increased activity of plasma and tissue kallikreins, plasma kininase II and salivary kallikrein in pemphigus foliaceus (fogo selvagem).

BACKGROUND: Pemphigus foliaceus (PF) is an autoimmune blistering disease of unknown aetiology, which is endemic in Brazil. Although the pathogenesis of PF is still unknown, proteins of the contact system have been implicated. OBJECTIVES: As the components of the kinin system may interact with those of the contact system, in this study we evaluated the plasma levels of high-molecular-weight kininogen (HK) and low-molecular-weight kininogen (LK), and the activity of plasma kallikrein, tissue kallikrein and kininase II in plasma of patients with PF presenting with Nikolsky's sign. As kidneys and salivary glands are relevant sources of tissue kallikrein for plasma, we also evaluated urinary/salivary kallikrein and urinary kininase II activities. METHODS: Fifteen patients and 15 age- and sex-matched controls were studied. Kininogen levels were determined by enzyme-linked immunosorbent assay, and the activities of kallikreins and kininase II were determined using selective chromogenic substrates. RESULTS: Compared with controls, plasma HK levels were decreased (P = 0.031), whereas the activities of plasma kallikrein, tissue kallikrein and kininase II in plasma, and the activity of salivary kallikrein, were increased in patients (P < 0.001 for each comparison). Plasma levels of LK and the activities of urinary kallikrein and urinary kininase II were not significantly different from controls. CONCLUSIONS: Diminished levels of HK associated with increased activities of plasma kallikrein and kininase II indicate that the kinin system is activated at the systemic level in PF. As active plasma kallikreins may act on some proteins of the contact system, it is possible that the enzyme may contribute to blister formation. The further observation of an increased tissue kallikrein activity at the systemic and saliva levels may be interpreted as a systemic reflex of skin inflammation. Whether the activation of the kinin system is a cause or a consequence of blister formation needs further clarification.

Adolescent↗
PubMed2003

Serum lipids of pemphigus foliaceus patients on long-term glucocorticoid therapy.

Endemic pemphigus foliaceus, and long-term corticotherapy may affect serum lipid levels. The aim of this study was to compare serum lipids of pemphigus foliaceus patients on glucocorticoid therapy to a healthy control group. Fifteen patients receiving prednisone (0.33 +/- 0.22 mg/kg) for at least 12 months and 15 controls were submitted to 48-h food intake records, anthropometry, and biochemical measurements. Data were compared by chi2, Mann-Whitney and Student "t" tests. The groups were matched for gender, age, weight, body mass index, arm circumference and triceps skin fold. No differences were observed in relation to energy, fat, protein and carbohydrate daily intakes, total cholesterol, HDL, LDL, uric acid, and serum creatinine levels. Pemphigus foliaceus patients had higher triglyceride [159 (64-371) vs. 100 (45-133) mg/dl], VLDL [32 (13-74) vs. 20 (9-114) mg/dl] and ESR [44 (9-87) vs. 7 (1-30) mm/h] levels than controls, probably due to metabolic effects of inflammatory disease and corticotherapy.

Blood Sedimentation↗
PubMed2026

When Neurodevelopment Meets Autoimmunity: Pemphigus Foliaceus in Rett Syndrome Expands the Clinical Spectrum-A Case Report.

Rett syndrome (RTT, OMIM 312750) is a complex multisystem neurodevelopmental disorder. Evidence suggests that RTT may have an autoimmune component and inflammatory activation. However, the autoimmune manifestations remain poorly described. Pemphigus foliaceus is a debilitating autoimmune blistering condition caused by IgG autoantibodies that target desmoglein-1 (Dsg1), resulting in widespread skin blistering and lesions. We report a case of pemphigus foliaceus in a 20-year-old female with RTT and discuss its clinical implications. Clinical data obtained from electronic health records were extracted and reviewed. Genetic testing was performed to identify the specific methyl-CpG-binding protein 2 (MECP2) mutation and on an expanded panel of 55 genes associated with pemphigus foliaceus and related blistering disorders. The individual had pemphigus foliaceus, which required immunosuppression, intravenous immunoglobulin (IVIg) therapy, and Rituximab. The disease trajectory was complicated by infections, aspiration pneumonia, and hypoxic cardiac arrest. There was progressive functional decline, and disease control was difficult to achieve, with frequent flares. Genetic testing confirmed a heterozygous pathogenic MECP2 variant (NM_001110792.1:c.952C>T; p.(Arg318Cys)). HLA genotyping identified alleles consistent with the HLA-DRB1*04:02-HLA-DQA1*03:01-HLA-DQB1*03:02 (DR4/DQ8) haplotype. Furthermore, genetic analysis identified a heterozygous DSG1 variant rs12967407. This study reports the first case of pemphigus foliaceus in RTT, expanding the clinical spectrum of RTT beyond its neurodevelopmental phenotype. The DR4/DQ8 haplotype, previously associated with pemphigus susceptibility, supports a background of genetic susceptibility in this individual. No causal association between RTT and pemphigus foliaceus can be inferred from this single case. Rather, this case demonstrates that a rare autoimmune disorder such as pemphigus foliaceus can co-occur with a pathogenic MECP2 mutation. The coexistence of a genetic and autoimmune disease can result in a more complex clinical presentation and treatment course. The case further emphasises the need for increased vigilance in identifying new and emerging systemic pathology alongside RTT.

Humans↗
PubMed2004

Prolonged remission after immunosuppressive therapy in six dogs with pemphigus foliaceus.

Limited information is available on the long-term outcome of treatment of pemphigus foliaceus in dogs. The purpose of this study is to report that a prolonged remission can occur after discontinuation of immunosuppressive regimens in some animals with this disease. Six dogs were diagnosed with pemphigus foliaceus based on suggestive clinical signs and histopathology. These patients were treated either with immunosuppressive doses of oral glucocorticoids or with a combination of oral glucocorticoids and azathioprine. After clinical signs underwent complete remission, which occurred 1.5-5 months after immunosuppression was initiated, the drugs were tapered progressively and eventually withdrawn. The total duration of immunosuppressive therapy varied between 3 and 22 months. Skin lesions of pemphigus foliaceus did not recur for 1.5-6 years after treatment was stopped. These observations suggest that, in some dogs with pemphigus foliaceus, immunosuppression can lead to long-term remission of skin lesions, and that discontinuation of treatment is not necessarily followed by a recurrence of clinical signs.

Animals↗
PubMed2000

The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research.

BACKGROUND: Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limão Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the disease, with a prevalence of 3.4 percent in the population. We tested the hypothesis that normal subjects living in an endemic area have antibodies against desmoglein 1. METHODS: We used an enzyme-linked immunosorbent assay to detect antibodies against desmoglein 1 in serum samples from 60 patients with endemic pemphigus foliaceus (fogo selvagem) who lived in Limão Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limão Verde and surrounding locations, and 126 normal subjects from the United States and Japan. RESULTS: Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 percent). Antibodies were also detected in 51 of the 93 normal subjects from Limão Verde (55 percent) and in 54 of the 279 normal subjects from surrounding areas (19 percent). Serum samples obtained one to four years before the onset of disease were available for five patients; all five had antibodies in the initial serum samples, and the onset of disease was associated with a marked increase in antibody values. CONCLUSIONS: The prevalence of antibodies against desmoglein 1 is high among normal subjects living in an area among where fogo selvagem is endemic, and the onset of the disease is preceded by a sustained antibody response. These findings support the concept that the production of antibodies against desmoglein 1 is initiated by exposure to an unknown environmental agent.

Autoantibodies↗