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PubMed2003

Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia.

BACKGROUND: We previously described a new focus of endemic pemphigus foliaceus in rural areas of El Bagre, Colombia, with clinical and direct immunofluorescence characteristics of pemphigus erythematosus. OBJECTIVE: The aim of this study was to characterize autoantigen profiles for 34 serum samples obtained from patients with this condition. METHODS: Immunofluorescence, various immunoblot analyses with different antigen sources and detection methods, and immunoprecipitation were performed. RESULTS: Immunofluorescence with the use of human skin sections showed IgG autoantibodies against keratinocyte cell surfaces in all 34 serum samples. Some samples also showed weak reactivity with the basement membrane zone. The results of immunoblot and immunoprecipitation analysis indicated that all sera had antibodies reactive with desmoglein 1, the pemphigus foliaceus antigen. In addition, in various immunoblot assays, many sera reacted with several other proteins with molecular weights of 250 kd, 210 kd, and 190 kd, which appear to correspond to desmoplakin I, envoplakin, and periplakin, respectively. CONCLUSION: This endemic pemphigus disease in El Bagre showed immunologic features similar to pemphigus foliaceus or erythematosus. In addition, paraneoplastic pemphigus-like reactivity with various epidermal antigens was detected.

Autoantigens↗

PubMed1993

Pemphigus foliaceus in young women. An endemic focus in the Sousse area of Tunisia.

BACKGROUND AND DESIGN: With the exception of some areas in Brazil, pemphigus is an exceptional disease. Several previous observations suggested that pemphigus foliaceus occurred more frequently than expected in Tunisia. To confirm these observations, a 15-month prospective survey was undertaken at the University Hospital of Sousse in Tunisia. RESULTS: During the survey, 23 patients with pemphigus were observed. Twenty of 23 cases were of pemphigus foliaceus that affected only young women. Seven patients had the clinical features of pemphigus herpetiformis. The estimated incidence of pemphigus foliaceus in the Sousse area was four new cases per million inhabitants per year, far above European or North American incidence but lower than the incidence in the most severely affected areas in Brazil. The high prevalence of the herpetiform clinical variant, the young age, the feminine exclusivity, and the absence of familial cases differentiated this Tunisian pemphigus both from pemphigus foliaceus observed in Europe and North America and from the Brazilian fogo selvagem. No etiologic factor was identified. CONCLUSIONS: This study suggests the existence in Tunisia (and possibly in all North Africa) of an endemic form of pemphigus foliaceus restricted to young women.

Adult↗

PubMed1982

Pemphigus foliaceus: a case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities.

The two variants of superficial pemphigus, pemphigus erythematosus (Senear-Usher syndrome) and pemphigus foliaceus, share common histopathologic and indirect immunofluorescence findings. They differ in that pemphigus erythematosus is limited in distribution and usually has concomitant basement membrane zone deposition of immunoglobulin and complement in lesional skin in addition to intercellular space staining in the epidermis, whereas pemphigus foliaceus is a generalized process that reputedly lacks basement membrane zone staining. A 49-year-old man with an array of immunologic abnormalities, including pernicious anemia and Sjögren's syndrome consistent with broad immunologic dysfunction, developed generalized superficial pemphigus (pemphigus foliaceus) with concomitant basement membrane zone immunoreactant deposition. The demonstration of basement zone immunofluorescent staining lends additional support to the basic viewpoint that pemphigus erythematosus and pemphigus foliaceus are variable phenotypic expressions of the same basic disease process.

Autoimmune Diseases↗

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↗

PubMed2001

A Case of pemphigus foliaceus which occurred after five years of remission from pemphigus vulgaris.

A 77-year-old Japanese female developed pemphigus foliaceus (PF) after 5 years of remission from pemphigus vulgaris (PV). The patient had painful erosions in her mouth and flaccid blisters of the skin and was diagnosed as having PV, which responded well to corticosteroid treatment. She was then free from any lesion of PV for 5 years with a low dose of corticosteroid. Then she developed scaly erythematous lesions on the skin and was diagnosed as suffering from PF. Enzyme-linked immunosorbent assay (ELISA) using recombinant desmoglein 1 (Dsg-1) and Dsg-3 revealed that she had anti-Dsg-3 IgG in the PV stage, no antibodies during remission and anti-Dsg-1 IgG in the PF stage. These findings indicate that the target antigen was shifted from Dsg-3 to Dsg-1 along with the phenotype after a 5-year interval in this patient.

Adrenal Cortex Hormones↗

PubMed1985

Brazilian pemphigus foliaceus autoantibodies are pathogenic to BALB/c mice by passive transfer.

Brazilian pemphigus foliaceus (fogo selvagem) is a cutaneous blistering disease endemic to certain areas of South America that has distinctive epidemiologic features suggestive of an infectious disease transmitted by an insect vector. Patients with the disease have antiepithelial autoantibodies, both circulating in the serum and bound to lesional epidermis. In order to examine the possible pathogenic role of these autoantibodies, IgG from the sera of these patients was purified and injected into the peritoneum of neonatal BALB/c mice. Thirty-four of 46 mice (74%) receiving parenteral IgG fractions from these patients developed cutaneous lesions that were identical to the human disease by clinical, histologic, immunologic, and ultrastructural criteria. High-titer Brazilian pemphigus foliaceus sera produced lesions more consistently and rapidly than low-titer sera. When injections were discontinued, new lesions ceased to appear and old lesions resolved. The extent of disease correlated with the titer of human antiepithelial antibodies detected in the mouse serum (z less than 0.01). Similar concentrations of IgG fractions obtained from sera of unaffected Brazilians living in endemic areas and from American donors did not induce disease when injected into littermates. These results establish that the antiepithelial autoantibodies play an important role in the pathogenesis of the cutaneous lesions in Brazilian pemphigus foliaceus.

Animals↗

PubMed1976

Autoimmunity in patients with Pemphigus foliaceus.

The presence of conventional autoantibodies in the sera from 28 patients with Pemphigus foliaceus (South American pemphigus) was investigated. Gastric parietal cell antibody was found in six patients (21.4 per cent); and in two of them mitochondrial antibody was also detected. Antibody to smooth muscle was found in one patient. Based on the above results, the role of autoimmunity in Pemphigus foliaceus was discussed. Nuclear and rheumatoid factors were not detected, and antibodies against glomerular components, salivary duct and thyreoglobulin were not found.

Adult↗

PubMed1999

Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris.

Patients with pemphigus foliaceus (PF) have blisters on skin, but not mucous membranes, whereas patients with pemphigus vulgaris (PV) develop blisters on mucous membranes and/or skin. PF and PV blisters are due to loss of keratinocyte cell-cell adhesion in the superficial and deep epidermis, respectively. PF autoantibodies are directed against desmoglein (Dsg) 1; PV autoantibodies bind Dsg3 or both Dsg3 and Dsg1. In this study, we test the hypothesis that coexpression of Dsg1 and Dsg3 in keratinocytes protects against pathology due to antibody-induced dysfunction of either one alone. Using passive transfer of pemphigus IgG to normal and DSG3(null) neonatal mice, we show that in the areas of epidermis and mucous membrane that coexpress Dsg1 and Dsg3, antibodies against either desmoglein alone do not cause spontaneous blisters, but antibodies against both do. In areas (such as superficial epidermis of normal mice) where Dsg1 without Dsg3 is expressed, anti-Dsg1 antibodies alone can cause blisters. Thus, the anti-desmoglein antibody profiles in pemphigus sera and the normal tissue distributions of Dsg1 and Dsg3 determine the sites of blister formation. These studies suggest that pemphigus autoantibodies inhibit the adhesive function of desmoglein proteins, and demonstrate that either Dsg1 or Dsg3 alone is sufficient to maintain keratinocyte adhesion.

Animals↗

PubMed2006

Pemphigus foliaceus induced by radiotherapy and responsive to dapsone.

Pemphigus foliaceus induced by ionizing radiation therapy is a rare condition. We describe the case of a 70-year-old female who developed pemphigus foliaceus after X-ray treatment for an adenocarcinoma of the left breast. The eruption started at the portal of irradiation and only subsequently spread to other cutaneous areas. Mucosal membranes were not affected. Skin lesions were completely responsive to dapsone therapy.

Adenocarcinoma↗

PubMed2002

Pemphigus foliaceus presenting as eruptive seborrheic keratosis and responding to oral gold treatment.

Pemphigus Foliaceus (PF) is an antibody-mediated autoimmune disease. IgG directed against desmoglein-1 induces acantholysis in the superficial epidermis, leading to the classic presentation of crusted erosions in a seborrheic distribution. We report a case of a 51-year-old African-American man with an 8-year history of PF, who developed multiple hyperpigmented, 'stuck-on' appearing verrucous papules and plaques on the back, chest, and neck. Skin biopsy and direct immunofluorescence from the seborrheic keratosis-like lesions was consistent with pemphigus foliaceus. The patient was treated by adding oral gold (auranofin) to his regimen of prednisone and discontinuing hydroxychloroquine. After six months of follow-up his erosions healed. This is the first report of treating pemphigus foliaceus with oral gold.

Auranofin↗

PubMed1983

Pemphigus foliaceus and proteinuria induced by alpha-mercaptopropionylglycine.

Pemphigus foliaceus and proteinuria occurred in a patient with cystinuria treated with alpha-mercaptopropionylglycine. This drug is similar to D-penicillamine in structure and function, and it is being used in patients who are unable to tolerate D-penicillamine. Although side effects of D-penicillamine are common, no major adverse reactions have been reported previously with alpha-mercaptopropionylglycine. Pemphigus foliaceus and proteinuria occurring during therapy with alpha-mercaptopropionylglycine and D-penicillamine are discussed.

Amino Acids, Sulfur↗

PubMed1994

Pemphigus foliaceus sera recognize an N-terminal fragment of bovine desmoglein 1.

It has previously been demonstrated that sera from endemic and nonendemic pemphigus foliaceus patients recognize three immunoreactive fragments of 80, 62, and 45 kilodaltons (kD) from extracts of the envelope fraction of human and bovine epidermis. These polypeptides are also immunoprecipitated by approximately 50% of pemphigus vulgaris sera, but are unreactive with sera from bullous pemphigoid patients or normal controls. The 80-kDa antigen has been shown to be a glycoprotein with N-linked oligosaccharides. Complete removal of the carbohydrate moieties produced a 76-kD polypeptide that continued to react with pemphigus foliaceus autoantibodies in a Ca(++)-dependent manner. To further characterize this antigen/antibody system, the 80-kD pemphigus foliaceus antigen solubilized from a bovine epidermal envelope extract was purified by affinity chromatography using a pemphigus foliaceus patient's immunoglobulin (Ig)G immobilized on agarose. After elution with 0.2 M glycine/HCl, pH 2.8, 5 mM ethylene diaminetetraacetic acid, the polypeptide was mixed with a small amount of 125I-labeled 80-kD antigen, added as a tracer, fractionated by sodium dodecylsulfate-polyacrylamide gel electrophoresis, and electrotransferred onto a polyvinylidene difluoride membrane. The 80-kD band detected by amido black staining and autoradiography was excised and characterized by amino acid sequence analysis. The resulting sequence, EXIKFAAAXREGEXNSKRNPIA, matched perfectly with the N-terminal 22 amino acids of the mature form of bovine desmoglein 1. These findings demonstrate that the 80-kD bovine autoantibody-reactive polypeptide is the glycosylated ectodomain of desmoglein 1, which may contain epitopes recognized by pathogenic autoantibodies.

Amino Acid Sequence↗

PubMed1993

Endemic pemphigus foliaceus. Fogo selvagem.

Fogo selvagem is a form of pemphigus foliaceus indistinguishable on clinical, histologic, immunofluorescence, and ultrastructural grounds from sporadic pemphigus foliaceus found in North America and Europe. It can, however, be distinguished on epidemiologic grounds by its unique geographic distribution. The association of FS with its environment is supported by its limited areas of predilection, that is, between 500 and 800 meters altitude near rivers, and the remission of the disease when patients are removed from this environment. Owing to its obvious environmental trigger, FS may serve as a model for the initiation of autoimmune phenomena. We need merely to find the specific trigger or triggers, and the search is now under way.

Brazil↗

PubMed2004

Pemphigus foliaceus in the horse: a retrospective study of 20 cases.

Twenty horses with pemphigus foliaceus were seen over a period of 15 years in a veterinary medical teaching hospital. Breeds seen were seven quarterhorses, five thoroughbreds, three cross-bred horses, two Arabians and one of each of the following: standardbred, Tennessee walker and warmblood. There was no breed, age or sex predisposition. Nine were mares, ten were geldings and one was a stallion. Ages ranged from 2.5 months to 25 years, with a mean of 8.6 years. Sixteen (80%) of the pemphigus foliaceus horses first exhibited signs between September and February. There was a statistically significant more common occurrence of pemphigus foliaceus during those months. Signs in the four other horses were first noted in March, May or June. Three of those horses were < 13 months of age. Oedema (14/20) and crusts (13/20) were the most common lesions. Pain was present in 9/20 horses, pruritus in 7/20 and pyrexia in 7/20. Follow-up was available for 13 horses. Five of these horses were euthanased. In three horses the reason for euthanasia was laminitis secondary to treatment. Four horses remained lesion-free after medication was discontinued. Two horses required maintenance medication and are doing well at the time of writing.

Animals↗

PubMed2001

Immunopathologic characterization of the tissue response in endemic pemphigus foliaceus (fogo selvagem).

BACKGROUND: The research on endemic pemphigus foliaceus (fogo selvagem) has mainly focused on the humoral immune response, but little attention has been given to the function of cell-mediated immune response and the nature of the cellular elements of the tissue reaction in the lesions of fogo selvagem. OBJECTIVE: The purpose of this study was the immunophenotype characterization of the inflammatory cells as well as the expression of adhesion molecules and HLA-DR in the perilesional and lesional skin of fogo selvagem. METHODS: Twenty biopsy specimens of lesional and perilesional skin were analyzed by immunohistochemical techniques. The panel of monoclonal antibodies consisted of CD8, CD4, CD1a, HLA-DR, IL-2R, LFA-1, ICAM-1, and PAN-B. RESULTS: The semiquantitative analysis of the cell population revealed a predominance of CD4 T lymphocytes in the tissue response of perilesional and lesional skin. The population of epidermal Langerhans cells was decreased in lesional skin when compared with the perilesional skin, whereas CD1a(+) dermal dendritic cells predominated in lesional skin. Keratinocyte expression of ICAM-1 and HLA-DR was negative in both lesional and perilesional skin. CONCLUSION: The overall results suggest the participation of the cell-mediated immunity in endemic pemphigus foliaceus (fogo selvagem). The lack of keratinocyte ICAM-1 expression may be related to the pattern of cytokines secreted by the CD4(+) T cells of the tissue reaction in fogo selvagem.

Antibodies, Monoclonal↗

PubMed1996

High-titer interferon-alpha antibodies in a patient with pemphigus foliaceus.

Among 13 patients with pemphigus or bullous pemphigoid, high titers of anti-interferon-alpha (IFN-alpha) antibodies were present in all serum samples of one patient suffering from pemphigus foliaceus. This patient was characterized by a relatively benign course of the disease. The IFN antibodies were of oligoclonal or polyclonal origin, predominantly of the IgG subtype, and displayed a broad spectrum of specificity including various natural and recombinant IFN-alpha subtypes as well as recombinant IFN-omega 1. In vitro, these antibodies neutralized both the antiviral and antiproliferative activities of the respective IFN types. Recognition of the patient's endogenous IFN-alpha demonstrated their autoantibody nature. The IFN antibodies were present at diagnosis and resistant to continued immunosuppressive treatment. Despite clinical remission, the IFN antibodies persisted, suggesting that they were not pathogenically related to the skin manifestations of the pemphigus. There were no sings of immune complex-mediated organ damage. IFN antibodies constitute a new class of autoantibodies that may occur in conjunction with pemphigus and likely interfere with the endogenous IFN system.

Adult↗

PubMed1991

Characterization of pemphigus foliaceus antigen from human epidermis.

Pemphigus foliaceus (PF) and its endemic form, Fogo Selvagem (FS), are characterized by subcorneal vesicles and pathogenic IgG autoantibodies directed against keratinocyte surface antigens. A major pool of FS antigen(s) remains bound to the insoluble epidermal envelope fraction. In this paper we demonstrate that this antigen(s) can be released from the envelope fraction by sonication. By immune precipitation four components can be detected, having molecular weights (MW) of 260, 80, 62, and 45 kD. The 260-kD component is lost by boiling or extraction with glycine HCl at pH 2.8. The major components appear to be the 80- and 62-kD poly-peptides. They chromatograph as a unit by gel filtration in 0.1% SDS, in the MW range of 115-120 kD. The FS antigen(s) appears to be cationic, forming insoluble complexes at low pH with SDS, and is labile to ammonium sulfate and freezing and thawing. It is unaffected by positive pressure concentration, 50% acetone precipitation, and reduction/alkylation. The FS antigen(s) is precipitated by all FS and nonendemic PF sera except those in complete clinical and serologic remission. The FS antigen(s) is also precipitated by 50% of pemphigus vulgaris but none of the bullous pemphigoid sera tested. All FS antigenic components are immunoprecipitated by IgG4 autoantibodies, but the IgG1 subclass from the same patients appear to immunoprecipitate only the 62-kD polypeptide. The FS antigen(s) is able to adsorb human autoantibodies against human desmoglein 1 (DG1), but not rabbit antisera against bovine DG1 or 2. This paper shows that physical stress, i.e., sonication, may be able to solubilize sufficient FS antigen(s) from the epidermal envelope fractions for further chemical characterization. The relationship of these FS antigen(s) to other reported FS antigens is presently unknown.

Autoantigens↗

PubMedDate not supplied

Treatment of pemphigus vulgaris and pemphigus foliaceus: experience with 71 patients over a 20 year period.

Forty one cases of pemphigus vulgaris and thirty cases of pemphigus foliaceus were investigated at Hospital Universitário Clementino Fraga Filho from 1978 to 1999. They were divided into two treatment groups: one group received up to 100 mg of oral prednisone daily and the other group received >120 mg daily. The dose up to 100 mg provided good initial control of pemphigus vulgaris and pemphigus foliaceus and did not increase the mortality rate associated to disease. The dose >120 mg induced higher morbidity. These data allowed us to establish a regimen of oral prednisone (1-2 mg/kg/daily) with maximum of 120 mg daily in the treatment of pemphigus vulgaris and pemphigus foliaceus.

Adolescent↗