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PubMed1993

Pemphigus foliaceus in a cat.

The author's cat started to develop the signs of pemphigus foliaceus one month after he returned home after six months absence. The initial signs included dry coughing and difficulty with purring and swallowing, followed by typical changes of the skin. The cat was treated by a combination of chrysotherapy and systemic glucocorticoid injections, and remained free of clinical signs for one and a half years. The cat then relapsed and showed the initial signs except that coughing was not observed. It was treated as before but after a second relapse and the same treatment it slowly developed a general weakness and was euthanased.

Animals↗
PubMed1989

Isolation of Cryptococcus albidus var. albidus in patient with pemphigus foliaceus.

Cryptococcus albidus var. albidus was isolated from the blood of a patient with pemphigus foliaceus after steroid therapy. This organism was found in triple extract peptone medium which is used in our laboratory for blood culture to detect aerobic and anaerobic bacteria. Identification of Cryptococcus was made by the API 20C yeast carbohydrate assimilation test, together with conventional procedures. These include the demonstration of chlamydospore production, germ tube test, urease test, nitrate assimilation test and colony morphology. The patient infected with Cryptococcus albidus var albidus had a good response to oral ketoconazole therapy, then the recovered and was discharged. The isolate obtained from this case may be regarded as an etiologic agent in fungemia.

Cryptococcosis↗
PubMed2004

Involvement of UV-irradiation in pemphigus foliaceus.

Ultraviolet (UV) light has been implicated in inducing acantholysis in the uninvolved skin of pemphigus patients. This report presents the case of a 55-year-old man with pemphigus foliaceus in whom skin lesions were induced and exacerbated by UV-irradiation.

Humans↗
PubMed1994

Shift in clinical features, histologic findings and antigen profiles from pemphigus vulgaris to pemphigus foliaceus--two case studies.

We investigated antigen profiles of the sera from 2 cases of pemphigus vulgaris (PV) who subsequently developed pemphigus foliaceus (PF). Immunoblotting was carried out using normal human epidermal extracts as the antigen source. The sera during PV reacted exclusively with the 130-kD PV antigen and the sera during PF exclusively with the 150-kD antigen. Therefore, dermatologic, histologic and immunohistologic findings before and after this transition were clearly shown related to changes in serum reactivity with pemphigus antigens.

Autoantigens↗
PubMed1997

Detection of canine pemphigus foliaceus autoantigen by immunoblotting.

The antigens targeted by autoantibodies in sera from canine patients with pemphigus foliaceus (PF) were detected by indirect immunofluorescence and Western immunoblotting. The extracted proteins from canine keratinocytes cultured in high-calcium condition for 48 h after confluency and from bovine nose epidermis were used as antigens in Western blotting. Canine keratinocytes cultured in high-calcium condition showed fluorescent deposits in intercellular spaces by incubation with sera from both canine and human pemphigus patients. By Western blotting, eight out of 16 canine PF sera recognised 160 kDa protein. 85 kDa and 120 kDa proteins were also recognised by four to five canine PF sera, respectively. The 160 kDa band, recognised by eight canine PF sera, had an identical mobility to the protein identified by a human PF serum. These results suggested that the autoantibodies in sera from canine PF recognised the 160 kDa desmosomal proteins, which may correspond to the desmoglein 1.

Animals↗
PubMed2005

Review of 15 cases of pemphigus foliaceus in horses and a survey of the literature.

The records of 15 horses with pemphigus foliaceus diagnosed on the basis of their history, clinical signs, histopathology and the exclusion of differential diagnoses were evaluated with respect to the age of onset, the clinical signs and the diagnostic tests used. There was no apparent breed predisposition. The horses' mean age was nine years, with a range from three months to 25.5 years, three were foals up to six months old and eight were nine years old or older. The most frequent lesions were scaling in 11, crusting in 10 and alopecia in 10, and they appeared most commonly on the face, neck and trunk, in 10 horses for each of these sites. The extremities were involved in nine of the horses, pruritus occurred in seven, and four of the horses had pustules. The clinical signs mostly corresponded with those described in previous reports, but signs of pain were not a prominent feature. Acantholytic cells were identified cytologically in four of six of the horses.

Animals↗
PubMed2005

The profile of fogo selvagem (endemic pemphigus foliaceus) at the University Hospital of Brasilia-Brazil. Epidemiological and clinical considerations.

BACKGROUND: Endemic pemphigus foliaceus (EPF) or fogo selvagem (FS) is an endemic autoimmune disease, characterized by flaccid bullae induced by IgG(4) subclass antibodies. The authors demonstrate the epidemiological and clinical status of patients who have been followed at the University Hospital of Brasilia (HUB) for more than 15 years. METHODS: One hundred and ninety-six patients with FS took part in the project. In setting up this study a historical descriptive cohort of patients was put together. In order to collect data, the authors used a questionnaire where the patient indicated the sex, age at the onset, occupation, origin, clinical status, including scalp compromise, evolution, cofactors influencing clinical worsening and the treatment compliance. In order to minimize loss to follow up, the authors used the statistical method of incidence density (patients/years). RESULTS: The disease occurred in 58.4% of the young patients in the 11-30-year age bracket, and 52% came from urban areas. These patients included students and teachers. Localized disease predominated as compared with the generalized presentation of this condition. Fifty-nine percent of the patients evolved to the recurrent form. Those patients with the evolutional form, in remission for more than 1 year (94%), had been followed for more than 5 years. Even patients with the less active forms of the disease had scalp lesions. Thus scalp lesions are not an indicator of bad prognosis. CONCLUSIONS: In the present study, the disease affected patients from a higher sociocultural class than previously described. Furthermore, in contrast to other reports, a substantial number of the patients lived in urban areas, although often spending some time in a rural setting for leisure or professional activities. This study suggests that the longer the follow up, the higher the likelihood that the disease would progress to a more controlled clinical presentation. Scalp lesions were not related to adverse prognosis. Sun, heat and infections act as triggering factors for the immunological imbalance, worsening the disease.

Adolescent↗
PubMed1993

[Pemphigus foliaceus caused by penicillamine].

We report the case of an 80-year-old woman who developed pemphigus foliaceus while taking penicillamine for rheumatoid arthritis. On the basis of this new case, we discuss fresh insights into the pathogenesis of drug-induced pemphigus. In addition, having reviewed the literature, we give a list of drugs that have been reported to induce this disease.

Aged↗
PubMed1992

Purification of immunoprecipitated pemphigus foliaceus antigen fragment and its use in radioimmunoassay.

A major difficulty in biochemical studies of the pemphigus foliaceus (PF) antigen is the lack of a method for its quantitative determination. Immunofluorescence blocking and immunoprecipitation methods are semiquantitative and time consuming. Radioimmunoassay (RIA) methods are quantitative but they require pure and stable antigen preparations that have not been available for PF. The present investigation shows the further purification of a previously described preparation of PF antigen fragment obtained from trypsinization media of mouse skin (Con A Frn) and demonstrates its usefulness in a RIA method for quantitation of the antigen. The major contaminants of the 45-kD tryptic fragment of the PF antigen (tf-PF) in immunoprecipitates of the Con A Frn with PF sera were identified as H and L chains of murine IgG and mannose-binding lectins. The IgG contaminants could be removed by avoidance of blood contamination during preparation of the Con A Frn and/or pre-absorption of the Con A Frn with protein A Sepharose. The lectins could be removed by affinity chromatography of the Con A Frn on asialofetuin column and washing the immunoprecipitates with 0.2 M alpha-methyl-mannoside. Using the purified, labeled Con A Frn in RIA, we showed that standard curves could be established and the amounts of PF antigen could be determined in different extracts without the need for electrophoresis, autoradiography, or scanning. This RIA method is rapid and can be easily used to analyze many samples, e.g., chromatographic fractions and extracts made from different tissues.

Animals↗
PubMed1982

Pemphigus foliaceus in a haemophilic child: cytomegalovirus induction?

A 3 1/2-year-old haemophilic child, repeatedly transfused with blood products, developed pemphigus foliaceus with presence in serum of both intercellular and anti-cytomegalovirus antibodies at high titre. The locus A26, which seems to be a genetic marker of pemphigus, was detected in his haplotype. Cytomegalovirus infection of transfusional origin is suspected to have triggered the outbreak of pemphigus.

Child, Preschool↗
PubMed1987

Substrate specificity of anti-epithelial antibodies of pemphigus vulgaris and pemphigus foliaceus sera in immunofluorescence tests on monkey and guinea pig esophagus sections.

The indirect immunofluorescent (IF) reactivity of the pemphigus antibodies in sera of 21 cases of pemphigus vulgaris (PV), 15 cases of pemphigus foliaceus (PF), and 14 cases of Brazilian PF (BPF) was compared on 2 substrates, notably monkey esophagus (ME) sections and guinea pig esophagus (GPE) sections. The IF reactions of the pemphigus antibodies of PV could be distinguished from those of PF or BPF by differences in their reactivity on ME and GPE sections in 98% of the cases examined in this study. In most cases, the pemphigus antibodies of PV cases gave higher titers and stronger IF staining reactions on ME sections, while those of PF and BPF cases gave stronger reactions on GPE sections. In addition, most (13 of 21) PV sera react with the lowest 3-4 cell layers of ME sections, while most (13 of 15) PF sera failed to do so but did react with the upper layers of the sections. Importantly, in 8 of the 50 cases examined by IF, the choice of substrate affected the detectability of the pemphigus antibodies, i.e., 4 of 15 PF and 2 of 14 BPF sera reacted only with GPE and 2 of 21 PV sera reacted only on ME. These research findings point to the need for an evaluation of the combined use of ME and GPE in routine diagnostic studies of pemphigus antibodies.

Animals↗
PubMed1980

Childhood pemphigus foliaceus. Report of a case.

A case is reported of a 4-year-old black boy with pemphigus foliaceus. The patient is unusual because of age, sex, race, and distribution of lesions, Confirmation of diagnosis was made by both routine histopathology and direct immunofluorescence microscopy. The patient responded rapidly to prednisone therapy.

Black People↗
PubMedDate not supplied

Nutritonal evaluation of pemphigus foliaceus patients on long term glucocorticoid therapy.

Our objective was to compare food intake and nutritional status of Pemphigus Foliaceus patients (PG) on long term glucocorticoid therapy to a Control Group (CG). Fourteen PG female inpatients receiving prednisone (0.33 +/- 0.22mg/kg) for at least 12 months and twelve CG subjects were submitted to nutritional evaluation, including anthropometry, urinary creatinine determination and serum biochemical measurements, besides 48-h-based food intake records. Groups were compared by Chi-square, Mann-Whitney and "t" tests. PG patients and CG were paired, respectively, in relation to age (24.7 +/- 14.1 vs. 22.0 +/- 12.0 years), body mass index (25.8 +/- 6.4 vs. 24.0 +/- 5.6kg/m2), daily protein intake (132.9 +/- 49.8 vs. 95.2 +/- 58.9g), and serum albumin (median; range) (3.8; 3.5-4.1 vs. 3.8; 3.6-5.0g/dl). However, PG patients had lower height-creatinine index (64.8 +/- 17.6 vs. 90.1 +/- 33.4%), and higher daily energy (3080 +/- 1099 vs. 2187 +/- 702kcal) and carbohydrate (376.8 +/- 135.8 vs. 242.0 +/- 80.7g) intakes. Despite high food, protein and energy consumption, PG patients on long term glucocorticoid therapy had lower body muscle mass than controls, while showing high body fat stores. These findings are possibly related to combined metabolic effects of long term corticotherapy and inflammatory disease plus corticosteroid-induced increased appetite.

Adult↗
PubMed1987

Pemphigus foliaceus antibodies and a monoclonal antibody to desmoglein I demonstrate stratified squamous epithelial-specific epitopes of desmosomes.

Pemphigus foliaceus (PF) is a human autoimmune disease in which autoantibodies are directed against the cell surface of epidermal cells. Using an immunoblotting technique, we recently demonstrated that a subgroup of PF patients have autoantibodies to the desmosomal core glycoprotein, desmoglein I (DGI). There are desmosomes in all epithelia and in heart, yet PF affects only stratified squamous epithelia. One explanation for this finding might be that there are tissue-specific differences in desmosomes. Thus, to determine whether certain epitopes of DGI are tissue-restricted, we performed immunofluorescence studies on various monkey tissues with the following antibodies: a rabbit polyclonal antiserum against whole desmosomes, which demonstrated the desmosomes in all tissues tested; a mouse monoclonal antibody against DGI, MmDGI-1; and PF sera that bound DGI on immunoblotting (PF IB+). In addition, we tested the tissues with PF sera that did not bind DGI by immunoblotting (PF IB-) to determine if these sera were different from PF IB+ sera in their tissue specificity. PF IB+, PF IB-, and MmDGI-1 antibodies stained all stratified squamous epithelia tested, including skin, tongue, upper esophagus, conjunctiva, and cornea; however, they did not stain heart or any nonstratified squamous epithelia, including gall bladder, small intestine, liver, ureter, and bladder. These results indicate that there is tissue heterogeneity of desmosomes, and that epitopes on DGI defined by both PF antibodies and a monoclonal antibody are present only in stratified squamous epithelia. In addition, PF IB- sera had the same tissue specificity as PF IB+ sera. These results may partially explain why PF involves only stratified squamous epithelia.

Antibodies, Monoclonal↗
PubMed2005

[Situation of endemic pemphigus foliaceus in Mato Grosso do Sul, Brazil, 1990-1999].

307 cases were examined to characterize epidemiological aspects of endemic pemphigus foliaceus. The disease was predominantly identified in rural workers between 10 and 42 years old. Incidence was higher in the December, January, February, March and May (average: 30.7 cases/year). Our findings identified a corridor, coinciding with the state watershed.

Adolescent↗
PubMed1989

Endemic pemphigus foliaceus (Fogo Selvagem).

Fogo selvagem presents our specialty with a unique situation. The disease is fundamentally identical to the pemphigus foliaceus that we are familiar with in North America, but there is impressive epidemiologic and circumstantial evidence that an environmental factor is producing this highly specific autoimmune phenomenon. If one can define this factor and investigate the mechanisms operative in the promotion of the disease, one would develop major new insights into both the normal function of the immune system and the specific disease state. Implications for understanding other autoimmune diseases are also obvious. However, to overcome the difficult logistics of studying these patients by modern techniques presents a formidable task. Hopefully, with the cooperation of interested investigators in both Brazil and the United States, these problems can be overcome.

Brazil↗