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PubMed2006

No evidence for association of the TP53 12139 and the BAX-248 polymorphisms with endemic pemphigus foliaceus (fogo selvagem).

Pemphigus foliaceus (PF) is an autoimmune bullous epidermal disease, characterized by autoantibodies specific to the desmosomal protein desmoglein 1 (dsg1) and by acantholysis, the rupture of the cellular junctions among keratinocytes. Known also as fogo selvagem (wild fire) in Brazil, the disease has distinct epidemiological characteristics, being endemic in certain regions of South America. It is a multifactorial (complex) disease, with oligo- or polygenic disease susceptibility. In view of the previously reported evidences of a role for apoptosis dysregulation in pemphigus pathogenesis, we hypothesized that genetic variants of molecules participating in apoptosis may contribute to interindividual variation of susceptibility to PF. The TP53 12139(G,C) and the BAX-248(G,A) single nucleotide polymorphisms (SNP) were analysed in a genetic association study. The allelic, genotypic and allele carrier frequencies for these SNPs did not differ statistically between the patient and the control groups, for both the Euro- and the Afro-Brazilian population strata. The results of this study lead us to conclude that, although the TP53 and BAX alleles analysed differ functionally, this variation does not alter the functionality of the molecules in a way that would interfere with the development of the disease.

Gene Frequency↗

PubMed1995

Coexistence of myasthenia gravis and pemphigus foliaceus.

Myasthenia gravis and pemphigus are both considered to have an autoimmune basis. Although immunological and clinical studies have been performed on large numbers of patients with myasthenia gravis, the coexistence of myasthenia gravis and pemphigus foliaceus has rarely been described. We recently have the opportunity to study a 33-year-old female patient having both of these autoimmune diseases confirmed by various diagnostic methods. This rare coexistence of myasthenia gravis and pemphigus foliaceus has not been previously documented in Korea.

Adult↗

PubMed2000

Serum cytokines in patients with Brazilian pemphigus foliaceus (fogo selvagem).

Pemphigus foliaceus (PF) is characterized by acantholysis determined by IgG4 binding to desmoglein I, a 160-kDa desmosomal glycoprotein. To investigate the immunopathological aspects of Brazilian PF, we determined levels of serum cytokines in patients with PF. Twenty-five patients with PF and a control group consisting of 10 healthy individuals were studied. Serum IL-2, IL-4, IL-5, IL-10, IL-12 and IFN-gamma were measured in the two groups by ELISA. The median concentration of IL-2 was lower in PF patients compared to the control group (0.45 and 9.50 pg/ml, respectively), as also was the concentration of IL-4 (0.26 and 10.16 pg/ml, respectively). The same was observed for IL-5 (7.94 and 15.74 pg/ml, respectively) and for IFN-gamma (5.90 and 8.58 pg/ml, respectively). For IL-10 and IL-12, higher concentrations were observed in PF compared to the control group (IL-10: 24.76 and 20.92; IL-12: 2.92 and 1.17 pg/ml, respectively). Considering the Th1/Th2 paradigm, it seems that a Th2 profile, mainly represented by IL-10, predominates in PF.

Adolescent↗

PubMed1993

Affinity immunoblotting studies on the restriction of autoantibodies from endemic pemphigus foliaceus patients.

Endemic pemphigus foliaceus or Fogo Selvagem (FS) is an autoimmune blistering skin disease mediated by autoantibodies directed against components on the surface of subcorneal keratinocytes. All patients have high titers of these autoantibodies in the IgG4 subclass as determined by indirect immunofluorescence on frozen skin sections. In addition, patients may also have autoantibodies in other IgG subclasses, particularly IgG1, but the titers in these subclasses are significantly lower than those found in the IgG4 subclass. We have now found that in addition to isotype preference, autoantibodies from FS patients show clonal restriction as evidenced by oligoclonal banding on isoelectric focusing after probing with extracts from both human and bovine epidermis. Both IgG1 and IgG4 exhibit oligoclonal banding, but the distribution of these bands in the pH gradient differs for these two subclasses. Whereas the IgG4 oligoclonal bands are distributed throughout the IgG4 pH range, IgG1 banding appears to be concentrated in the more basic region of the IgG1 pH range. This finding suggests that the IgG1 autoantibodies have undergone selective somatic mutation by a negatively charged autoantigen. Similar findings have been reported for pathogenic DNA autoantibodies associated with SLE. The wide distribution of IgG4 banding suggests that this response may have followed the IgG1 response and has not undergone selective mutation. Both IgG1 and IgG4 appear to be Ca++ dependent autoantibodies.

Adult↗

PubMed2005

Decline of anti-desmoglein 1 IgG ELISA scores by withdrawal of D-penicillamine in drug-induced pemphigus foliaceus.

A case of pemphigus foliaceus arising during the administration of D-penicillamine was reported. A 72-year-old woman with systemic sclerosis developed flaccid vesicles and crusted erythema of the skin after administration of D-penicillamine for 1-year. She was positive for anti-desmoglein 1 IgG by ELISA. The withdrawal of D-penicillamine resulted in improvement of the skin lesions and ELISA scores for anti-demoglein 1 antibodies revealed a surprisingly rapid decline. This case is a classical incidence of drug-induced pemphigus foliaceus, in which D-penicillamine was constantly required not only to induce, but also to maintain the production of the autoantibodies.

Aged↗

PubMed2006

Polymorphism of the promoter region and exon 1 of the CTLA4 gene in endemic pemphigus foliaceus (fogo selvagem).

Endemic pemphigus foliaceus (EPF) is an autoimmune bullous skin disease characterized by acantholysis and antibodies against a desmosomal protein, desmoglein 1. Genetic and environmental factors contribute to development of this multifactorial disease. HLA class II and some cytokine gene polymorphisms are the only genetic markers thus far known to be associated with susceptibility to or protection from EPF. The cytotoxic T-lymphocyte antigen-4 gene (CTLA4) encodes a key immunoreceptor molecule that regulates and inhibits T-cell proliferation. It participates in the regulatory process controlling autoreactivity and therefore has been considered a strong candidate gene in autoimmune diseases. In the search for genes that might influence EPF pathogenesis, we analyzed variants of the CTLA4 gene in a sample of 118 patients and 291 controls from a Brazilian population. This is the first study investigating the possible role of polymorphisms of the 2q33 chromosomal region in differential susceptibility to pemphigus foliaceus. Promoter region and exon 1 single nucleotide polymorphisms -318 (C,T) and 49 (A,G) were genotyped using sequence-specific oligonucleotide probes after amplification by the polymerase chain reaction. The allelic and genotypic frequencies did not differ significantly between the patient and the control groups (-318T: 9.8 and 10.9%, 49G: 33.0 and 35.2% were the allelic frequencies in patients and controls, respectively). In addition, no significant difference was found when the patient and control population samples were stratified by the presence of HLA-DRB1 alleles. We conclude that the CTLA4 -318 (C,T) and 49 (A,G) polymorphisms do not play a major role in EPF development.

Antigens, CD↗

PubMedDate not supplied

Evaluation of nutritional status in patients with endemic pemphigus foliaceus.

Sixteen patients with endemic pemphigus foliaceus were submitted to nutritional evaluation. Ten had the localized form of the disease (Group G1) and six the disseminated form (Group G2). The patients were submitted to anthropometric measurements (weight, height, Quetelét index, tricipital skin fold, subscapular skin fold, arm circumference, arm muscle circumference, arm area, arm muscle area, and arm adipose area) and to laboratory evaluation by protein electrophoresis. Arm circumference, arm area and arm muscle area showed lower values in G2 than in G1. Weight and arm muscle circumference tended to the lower in G2 than in G1. Protein electrophoresis showed decreased albumin levels in both groups, with lower values in G2. Overall analysis of the results permits us to conclude that patients with endemic pemphigus foliaceus present signs and symptoms of protein, but not calorie, malnutrition and that this malnutrition is more marked in patients with disseminated pemphigus foliaceus.

Adolescent↗

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

Kaposi's sarcoma after steroid therapy for pemphigus foliaceus.

A patient with pemphigus foliaceus, derived from pemphigus erythematosus, developed Kaposi's sarcoma characterized by wide-spread skin nodules on the extremities after 5-month treatment with large doses of prednisone. The patient died one year later. We conclude that the immunosuppressive treatment with prolonged large doses of prednisone is attributable to the impairment of cellular immunity and the evolution of Kaposi's sarcoma.

Adult↗

PubMed2003

230-kDa and 190-kDa proteins in addition to desmoglein 1 as immunological targets in a subset of pemphigus foliaceus with a combined cell-surface and basement membrane zone immune staining pattern.

Pemphigus erythematosus, initially described as a combination of pemphigus with lupus erythematosus, and pemphigus foliaceus are now frequently considered localized and generalized variants of superficial pemphigus. Yet diagnostic criteria for pemphigus erythematosus remain controversial. Distinct from pemphigus foliaceus, pemphigus erythematosus displays immune depositions at the dermal-epidermal junction, which suggests additional immunopathological mechanisms. We present three patients with clinical and histopathologic signs of superficial pemphigus, who all exhibited an immunomorphology characteristic of pemphigus erythematosus. Complement depositions in a granular-linear fashion were consistently found at the dermal-epidermal junction besides in vivo bound and circulating antikeratinocyte cell-surface autoantibodies. Histopathology showed subcorneal acantholysis, and all sera contained antidesmoglein 1 but not antidesmoglein 3 autoantibodies detected by enzyme-linked immunosorbent assays (ELISA). Additional autoantibodies against a 230-kDa protein and against a 190-kDa protein comigrating with bullous pemphigoid antigen 1 (BP230) and periplakin, respectively, were present in all the patients' sera. As two sera specifically reacted with BP230 by ELISA, the presence of BP230-specific autoantibodies could be associated with dermal-epidermal immune staining in these patients. In pemphigus erythematosus, dermal-epidermal immune staining is generally attributed to the deposition of immune complexes, while the presence of BP230-specific autoantibodies has not been reported in this disease previously. Perhaps, the unique autoantibody profile of the patients in the study permits discrimination between patients with superficial pemphigus that display additional dermal-epidermal immune staining from those with conventional pemphigus foliaceus on a molecular basis. Further studies will be required to substantiate the frequency of this occurrence and to unravel its pathogenic significance.

Adult↗

PubMed1987

'Tin-tack' sign in localized pemphigus foliaceus.

Two patients with localized pemphigus foliaceus are described, in whom a positive 'tin-tack' sign was a significant feature; this is the appearance of small horny plugs attached to the undersurface of the scale removed from the affected site. This report is intended as a reminder that a positive 'tin-tack' sign is not exclusively a feature of discoid lupus erythematosus but is also seen in localized pemphigus foliaceus.

Adult↗

PubMed2001

Nonendemic pemphigus foliaceus presenting as fatal bullous exfoliative erythroderma.

Pemphigus foliaceus is a cutaneous autoimmune blistering disease that is characterized by lower morbidity and mortality than those observed in pemphigus vulgaris or paraneoplastic pemphigus. However, erythrodermic forms of the endemic variant of pemphigus foliaceus have been associated with a higher mortality. We report a case of nonendemic pemphigus foliaceus that presented as fatal bullous exfoliative erythroderma, and thus, we will emphasize the inclusion of this entity in the differential diagnosis and the use of skin direct immunofluorescence in the evaluation of patients with erythroderma.

Aged↗

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↗

PubMed1999

Pemphigus vulgaris and pemphigus foliaceus antibodies are pathogenic in plasminogen activator knockout mice.

Previous studies have suggested that urokinase plasminogen activator is required for blister formation in pemphigus vulgaris and pemphigus foliaceus. Other studies, however, have shown that downregulation of plasminogen activator does not inhibit blisters induced by pemphigus immunoglobulin G. To eliminate the possibility that small amounts of urokinase plasminogen activator might be sufficient for blister formation, we passively transferred pemphigus immunoglobulin G to urokinase plasminogen activator knockout neonatal mice. Pemphigus foliaceus and pemphigus vulgaris immunoglobulin G caused gross blisters and acantholysis in the superficial and suprabasal epidermis, respectively, to the same degree in knockout and control mice, demonstrating that urokinase plasminogen activator is not absolutely required for antibody-induced blisters. Some studies have shown elevated tissue-type plasminogen activator in pemphigus lesions. Tissue-type plasminogen activator, however, is not necessary for blister formation, because pemphigus foliaceus and pemphigus vulgaris immunoglobulin G caused blisters to the same degree in tissue-type plasminogen activator knockout and control mice. To rule out that one plasminogen activator might compensate for the other in the knockout mice, we bred urokinase plasminogen activator, tissue-type plasminogen activator double knockouts. After passive transfer of pemphigus foliaceus and pemphigus vulgaris immunoglobulin G these mice blistered to the same degree as the single knockout and control mice, and histology indicated blisters at the expected level of the epidermis. These data definitively demonstrate that plasminogen activator is not necessary for pemphigus immunoglobulin G to induce acantholysis in the neonatal mouse model of pemphigus.

Animals↗

PubMedDate not supplied

Eyelid skin involvement in pemphigus foliaceus.

PURPOSE: To report the involvement of eyelid skin in a patient with pemphigus foliaceus. METHODS: Retrospective, interventional case report of a patient with pemphigus foliaceus involving both eyelids. The diagnosis of pemphigus foliaceus was based on histopathology and confirmed by direct immunofluorescence and immunoblot. Failure to respond to conventional immunosuppressive therapy, including high-dose corticosteroids, methotrexate, azathioprine, dapsone, and mycophenolate mofetil, necessitated the use of intravenous immunoglobulin (IVIg) therapy. RESULTS: After successful treatment with IVIg, no change in visual acuity or other sequelae secondary to ocular pemphigus foliaceus were observed. Normal ocular architecture was maintained. Clinical remission was observed after successful therapy. CONCLUSIONS: Pemphigus foliaceus involves the skin of the eyelid and does not involve the conjunctiva. The patient did not respond to conventional immunosuppressive therapy. The use of IVIg as mono-therapy resulted in long-term clinical remission.

Adult↗

PubMed2001

Comparative study of pemphigus vulgaris and pemphigus foliaceus in Singapore.

This is a retrospective study of all patients diagnosed to have pemphigus in our centre over a 3 year period. The case records of all patients with pemphigus from January 1995 to December 1997 were analysed. Fifty patients were diagnosed to have pemphigus during the study period. The diagnoses were pemphigus vulgaris in 31 patients, pemphigus foliaceus in 16, paraneoplastic pemphigus in two and IgA pemphigus in one. The average titre of anti-intercellular antibodies in patients with pemphigus vulgaris (1:96) was higher than the titre in patients with pemphigus foliaceus (1:69). The average initial dose of prednisolone required for disease control in patients with pemphigus vulgaris (62 mg/day) was significantly higher than that required for patients with pemphigus foliaceus (44 mg/day). In our study population, pemphigus vulgaris is a more severe and chronic disease than pemphigus foliaceus, as reflected in the higher titre of anti-intercellular antibodies, higher dose of systemic corticosteroids required for control of the disease, the longer duration to achieve complete remission and longer follow-up period.

Adult↗

PubMed1997

Recognition of pemphigus antigens in drug-induced pemphigus vulgaris and pemphigus foliaceus.

BACKGROUND: The clinical appearance and biologic behavior of drug-induced pemphigus depend on the type of inducing drug. OBJECTIVE: Our purpose was to investigate patients with drug-induced pemphigus vulgaris and pemphigus foliaceus antigens and compare results of studies to detect antibody reactivity in sera of these patients with the serology of patients with idiopathic pemphigus. METHODS: Ten patients with drug-induced pemphigus were studied. Antibody reactivity was determined against the pemphigus vulgaris antigen, desmoglein 3, and against desmoglein 1. RESULTS: The patient with pemphigus foliaceus and low levels of autoantibodies precipitated neither antigen. One patient with pemphigus vulgaris and high levels of antibody also failed to precipitate any specific antigen. Sera from eight patients with drug-induced pemphigus vulgaris had circulating autoantibodies directed to either the pemphigus vulgaris or pemphigus foliaceus antigen. Low levels of antibody in two of these eight patients precipitated only the pemphigus foliaceus antigen. High levels of antibody in five of the eight patients precipitated the pemphigus vulgaris antigen; two of these also reacted with the pemphigus foliaceus antigen. CONCLUSION: The autoantibody response was similar in both spontaneous and drug-related disease. A similar molecular mechanism in the two types of pemphigus is suggested.

Adult↗

PubMed1994

Comparative histopathology of pemphigus foliaceus and superficial folliculitis in the dog.

A comparative histopathologic study of 50 cases of pemphigus foliaceus and 47 cases of superficial folliculitis in the dog was undertaken to identify those histopathologic features important in differentiating these diseases. All cases were diagnosed by the Surgical Pathology Service of the Laboratory of Pathology of the University of Pennsylvania, School of Veterinary Medicine, between January 1986 and March 1991. These cases were followed clinically to determine the accuracy of the initial histopathologic diagnosis. Data were analyzed by Fisher's exact test for discrete data and the Mann-Whitney U-test for continuous data. A probability of < 0.05 was considered statistically significant. The following breeds were affected with pemphigus foliaceus: Akita, Brittany Spaniel, Chow Chow, Collie, Dachshund, Doberman Pinscher, English Cocker Spaniel, English Setter, Golden Retriever, Siberian Husky, Laborador Retriever, Miniature Schnauzer, Old English Sheepdog, Scottish Terrier, Chinese Shar Pei, Spitz, Shetland Sheep Dog, Weimeraner, and West Highland White Terrier. An increased risk of developing pemphigus foliaceus was noted in the Akita (OR = 37.8), English Springer Spaniel (OR = 20.7), Chow Chow (OR = 12.3), Chinese Shar Pei (OR = 7.9), and Collie (OR = 3.9). Pemphigus foliaceus had a higher average density of acantholytic cells (226 +/- 22.9) than superficial folliculitis (11.8 +/- 4.6), and acantholytic cells were 183 times more likely to be present in pemphigus foliaceus. The presence of rafts of acantholytic cells occurred in pemphigus foliaceus (23/50) more often than superficial folliculitis (1/47). Recornification and reformation of pustules were, respectively, 13.4 and 3.6 times more likely to occur in pemphigus foliaceus.(ABSTRACT TRUNCATED AT 250 WORDS)

Acantholysis↗