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PubMed1995

Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women.

Recent studies have suggested that in Tunisia, pemphigus foliaceus is more frequent in young women than expected. To confirm these findings, we compared the incidence rates of pemphigus in Tunisia as a whole and in a large area of France. New cases of pemphigus were detected retrospectively from dermatology departments and pathology laboratories over a 6-year period and classified as pemphigus vulgaris or foliaceus according to the pathology. In France, the incidence rate was 1.7 cases per million per year (95% confidence interval 1.4 to 2.1). Pemphigus vulgaris accounted for 73% of all cases, incidence increased with age, and the female-to-male sex ratio was 1.2. The incidence rate was significantly higher in Tunisia than in France: 6.7 cases per million per year (95% confidence interval 5.8 to 7.7); pemphigus foliaceus was more frequent (61%), the female-to-male sex ratio was 4.1, and the incidence rate was higher in young women. The incidence rate was 15.5 cases per million per year for pemphigus foliaceus among women aged 25 to 34 years and was even higher in some rural areas. No case was observed among household members or in neonates, and only one case occurred in childhood. Thus, we confirmed that the epidemiology of pemphigus in Tunisia is unusual. High rates of pemphigus foliaceus among young people living in rural areas are reminiscent of Brazilian pemphigus. However, the absence of cases among genetically related household members and during childhood, and the large predominance of women, contrast with Brazilian pemphigus.

Adult↗
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↗
PubMed2001

Diagnostic features of pemphigus vulgaris in patients with pemphigus foliaceus: detection of both autoantibodies, long-term follow-up and treatment responses.

There are several studies that describe the simultaneous presence and conversion of pemphigus foliaceus into pemphigus vulgaris and vice versa. We describe eight patients with clinical, histological and immunopathological features of pemphigus foliaceus, at the time of the initial diagnosis. After a mean period of 2.5 years, additional serological features of pemphigus vulgaris were observed. During a long-term follow-up, systemic therapies, their durations and treatment outcomes were recorded. These patients did not respond to conventional systemic therapy and developed multiple side-effects from these drugs. Hence, they were treated with intravenous immunoglobulin therapy (IVIg). Prior to the initiation of IVIg therapy, different assays were performed to detect the presence of autoantibodies, including indirect immunofluorescence (IIF), immunoblot assay using bovine gingival lysate, and ELISA. Twenty-five healthy normal individuals, 12 patients with pemphigus vulgaris, and eight patients with pemphigus foliaceus served as controls for comparison of serological studies. At the time of initial diagnosis, the sera of all eight study patients also demonstrated binding on an immunoblot assay to a 160-kDa protein (desmoglein 1) only. This is typically observed in pemphigus foliaceus. Prior to staring IVIg therapy, binding was observed to both the 160 kDa and 130 kDa (desmoglein 3) proteins on an immunoblot assay which was characteristic of pemphigus vulgaris. The antidesmogleins, 1 and 3 autoantibodies, were predominantly of the IgG4 subclass in all eight patients studied. IVIg therapy induced remission in four patients and control in four of the eight patients. The total follow-up period ranged from 2.6 to 9.5 years (mean 5.3 years). It is difficult to determine the exact time at which these patients with pemphigus foliaceus developed pemphigus vulgaris. It is possible that the disease was nonresponsive to conventional immunosuppressive therapy owing to the simultaneous presence of two autoantibodies.

Adult↗
PubMed1989

Demonstration of an adhering-junction molecule (plakoglobin) in the autoantigens of pemphigus foliaceus and pemphigus vulgaris.

Pemphigus foliaceus and pemphigus vulgaris are skin diseases in which antibodies against the cell surface of keratinocytes destroy the adhesion between epidermal cells, producing blisters. Patients with pemphigus foliaceus have antibodies to a complex of three polypeptides of 260, 160, and 85 kd (the foliaceus complex), whereas patients with pemphigus vulgaris have antibodies to a complex of 210-kd, 130-kd, and 85-kd polypeptides (the vulgaris complex). The 160-kd polypeptide of the foliaceus complex has been identified as desmoglein, a desmosomal glycoprotein. We suspected that the 85-kd component in both these antigenic complexes might be plakoglobin, another molecule in the adhering junctions of cells. To characterize these antigenic complexes, we used the serum of five patients with pemphigus foliaceus, that of four patients with pemphigus vulgaris, and monoclonal antiplakoglobin antibodies. We found that monoclonal antibodies to plakoglobin immunoprecipitated the 85-kd polypeptide from the dissociated foliaceus and vulgaris complexes and precipitated both complexes from epidermal extracts. Serum from patients with pemphigus foliaceus or pemphigus vulgaris (but not from four normal controls) bound desmoglein and the 130-kd polypeptide, respectively, showing that these peptides (and not plakoglobin) are the antigenic binding sites in these disorders. We conclude that plakoglobin, a protein of the adhering junctions of epidermal cells, is the 85-kd molecule in the antigenic complexes found in both pemphigus foliaceus and pemphigus vulgaris, although it is not the binding site in either disorder.

Autoantibodies↗
PubMed1993

[Comparative study of the development and prognosis of pemphigus vulgaris and seborrheic pemphigus].

Traditionally, the prognosis of pemphigus erythematosus is thought to be more favourable than that of pemphigus vulgaris. A retrospective study of the records of 10 patients with pemphigus erythematosus and 13 patients with pemphigus vulgaris was set up to compare the courses of the two diseases. This comparison, carried out in populations with similar age, sex ratio, pretreatment duration of the disease and treatment received, showed that relapses were more frequent in the course of pemphigus erythematosus, whereas remissions, mean duration of the disease and iatrogenic complications were the same in both groups. This study, therefore, throws some doubts on the dogma of relative mildness of pemphigus erythematosus, which goes back to a period long before systemic corticosteroid therapy was known. Mortality studies performed since the event of this treatment have shown that the prognosis had improved and tended to be the same in both diseases. The other data concerning the course of treated pemphigus erythematosus are little known and were never compared with those concerning pemphigus vulgaris. Our study shows that treatments similar to those of pemphigus vulgaris are necessary to obtain remissions in pemphigus erythematosus and that these diseases share the same evolutive profile. However, the question of the best therapeutic strategy to be used has not yet been answered.

Adult↗
PubMed2003

Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil.

BACKGROUND: Mycophenolate mofetil is increasingly being used as a corticosteroid-sparing agent in immunosuppressive regimens. OBJECTIVE: To elucidate the effectiveness of mycophenolate as adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. DESIGN: Historical prospective study. SETTING: University hospital. PATIENTS: The study included 42 consecutive patients with pemphigus (31 with pemphigus vulgaris and 11 with pemphigus foliaceus) who had relapses during prednisone taper or had clinically significant adverse effects from previous drug therapy. RESULTS: Remission was achieved in 22 (71%) and 5 (45%) of patients with pemphigus vulgaris and pemphigus foliaceus, respectively. Partial remission was achieved in 1 (3%) and 4 (36%), respectively. The median time to achieve complete remission was 9 months (range, 1-13 months). The treatment was administered for a median of 22 months, and the median follow-up period was 22 months. Seventy-seven percent of patients had no adverse effect. Two patients had side effects severe enough to necessitate discontinuation of treatment, one because of symptomatic but reversible neutropenia and the other because of nausea. CONCLUSION: Mycophenolate is an effective and safe adjuvant in the treatment of both pemphigus vulgaris and pemphigus foliaceus.

Adolescent↗
PubMed2003

Detection of pemphigus autoantibodies by IIF and ELISA tests in patients with pemphigus vulgaris and foliaceus and in healthy relatives.

BACKGROUND: Pemphigus is a life-threatening, autoimmune blistering disease, mediated by IgG autoantibodies. The aim of our study was to assess the usefulness of a new enzyme-linked immunosorbent assay (ELISA) in detecting circulating pemphigus autoantibodies, and to compare its sensitivity and specificity with the indirect immunofluorescence (IIF) test. We also established the frequency of occurrence of pemphigus autoantibodies in relatives of our patients. MATERIAL/METHODS: IIF and ELISA tests were performed in 24 patients with pemphigus vulgaris, 13 with pemphigus foliaceus, 56 healthy relatives, and 50 controls, selected according to sex and age. RESULTS: The obtained results revealed high specificity and sensitivity of ELISA, comparable to the IIF test, especially in patients who were in the active stage of the disease. We also showed that the profile of anti-Dsg 1 and/or anti-Dsg 3 autoantibodies is associated with the clinical variant of pemphigus. The frequency of occurrence of pemphigus autoantibodies in the relatives (24/55) performed by IIF was significantly higher (p<0.001) than in the controls (0/50). The same antibodies detected by ELISA (11/56) were less frequent. CONCLUSIONS: In clinically doubtful cases, in which autoantibodies titres by IIF are equal on both substrates (monkey and guinea pig esophagus), the assessment of the exact antibody profile (anti-Dsg 1 and/or anti-Dsg 3) is important to establish the subtype of pemphigus. The frequency of pemphigus antibody occurrence in healthy relatives seems not to be incidental, but further studies should be performed to explain this phenomenon.

Adolescent↗
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↗
PubMed1986

Complement fixation by pemphigus antibody. III. Altered epidermal cell membrane integrity mediated by pemphigus antibody and complement.

The present study investigates the effects of pemphigus IgG and complement upon cell viability and/or membrane integrity using trypan blue exclusion, ethidium bromide (EB) staining, and fluorescein diacetate (FDA) conversion by living cells. Forty-eight-hour cultivated epidermal monolayers of neonatal BALB/c mice were incubated in media containing 1 mg/ml purified pemphigus IgG for 48 h in either the presence or absence of complement (absorbed AB sera). Adherent and detached cells were examined by both phase and fluorescence microscopy. Results from trypan blue exclusion showed that pemphigus IgG plus complement produced a modest decrease in exclusion of the dye compared to pemphigus IgG without complement. When FDA/EB comparisons were made, however, the differences were more substantial. When complement plus pemphigus IgG was added to cultures, the number of FDA-positive adherent cells decreased significantly and the number of EB-positive detached cells increased significantly. The effects of complement were inhibited by the use of heat-inactivated AB sera or by C1q depletion of AB sera. No significant effect on the cells was observed in the presence or absence of complement when pemphigus F(ab')2 fragments or when normal IgG was used. Plasminogen depletion of the complement source did not interfere with complement and pemphigus IgG effects as judged by the FDA/EB assay. These studies suggest that pemphigus antibody in the presence of complement alters cell membrane integrity and supports the contention that complement may play a significant role in the mechanism of acantholysis.

Animals↗
PubMed2004

Endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris: immunoglobulin G heterogeneity detected by indirect immunofluorescence.

UNLABELLED: Pemphigus are autoimmune intraepidermal blistering diseases in which immunoglobulin G (IgG) autoantibodies are directed against desmosomal glycoproteins. The aim of this study was to determine the IgG subclass profile of endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris utilizing indirect immunofluorescence. PATIENTS AND METHODS: Twenty-five patients with pemphigus vulgaris, 25 with endemic pemphigus foliaceus (fogo selvagem), and 25 healthy controls were analyzed by indirect immunofluorescence for circulating autoantibodies (total IgG and its subclasses). RESULTS: Our data revealed a significant correlation (P <.05) of disease activity and autoantibody levels in both forms of pemphigus, i.e., negative titers related to clinical remission, whereas positive results related to active disease. Immunoglobulin G subclass analysis in fogo selvagem demonstrated that in patients in remission, 56% showed positive immunoglobulin G4; in active disease, immunoglobulin G4 was the predominant subclass (100% positive in all cases). The IgG subclass profile in pemphigus vulgaris showed that in patients in remission, only 10% were positive for immunoglobulin G4; in active disease, positivity for immunoglobulin G4 was present in 78% to 88% of the cases. CONCLUSION: Subclass characterization of immunoglobulin G autoantibodies is a useful tool for pemphigus follow-up, since immunoglobulin G4 (IgG4) is the subclass that is closely related to recognition of pathogenic epitopes, and consequently with disease activity. Careful monitoring should be performed for fogo selvagem in clinical remission with a homogeneous IgG4 response, since this may indicate more frequent relapses.

Adult↗
PubMed1981

Pemphigus vulgaris. Superior sensitivity of monkey esophagus in the determination of pemphigus antibody.

This study demonstrates that monkey esophagus is a more sensitive substrate than guinea pig esophagus for the demonstration of serum antibodies, in patients with pemphigus vulgaris. In this study, for example, five patients with active pemphigus who had demonstrable pemphigus antibodies on monkey esophagus substrate failed to demonstrate pemphigus antibody activity when guinea pig esophagus was employed as a substrate. Furthermore, pemphigus antibody titers determined on monkey esophagus substrate frequently exceeded, by several dilutions, titers detected with guinea pig esophagus. By contrast, in no instance did we detect pemphigus antibody titer activity on guinea pig esophagus in excess of the pemphigus antibody activity detected on monkey esophagus. The reason for the disparity in the sensitivity of the guinea pig and monkey esophagus substrates is unknown.

Animals↗