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PubMed2004

Direct immunofluorescence on uninvolved, lesional and perilesional skin in patients with endemic pemphigus foliaceus (fogo selvagem).

BACKGROUND: Research on direct immunofluorescence in pemphigus foliaceus ("fogo selvagem") has been focused mainly on the study of perilesional and lesional skin, while little attention has been given to uninvolved skin. We analyzed the frequency of IgA, IgM, IgG and its subclasses (IgG1, IgG2, IgG3 and IgG4) and C3 complement fraction deposition in intercellular spaces (ICS) and basal membrane zone (BMZ) in uninvolved, lesional and perilesional skin from 47 fogo selvagem patients. MATERIAL/METHODS: For each patient biopsies were collected from lesional, perilesional and uninvolved skin, and then analyzed by direct immunofluorescence. The panel of antibodies consisted of IgA, IgM, IgG and its subclasses (IgG1, IgG2, IgG3 and IgG4) and C3 complement fraction. RESULTS: The results showed a predominance of IgG and IgG4 deposit in all skin samples, followed by C3 complement fraction and IgG1 deposits. The positive response for IgG on uninvolved (91.48%), lesional (93.61%) and perilesional (97.87%) skin was similar to that found for IgG4 in the same samples: 95.74%, 95.74% and 97.87%, respectively. Regarding IgG1, the uninvolved skin showed lower results (14.89%) than the lesional (29.78%) and perilesional skin (29.78%). Concerning C3 complement fraction, the perilesional skin showed higher results (40.42%) than the uninvolved and lesional skin (34.04% for both). CONCLUSIONS: The results suggest the importance of uninvolved skin for direct immunofluorescence in the diagnostics of pemphigus foliaceus. Our results suggest that any cutaneous region can demonstrate pemphigus antibodies by direct immunofluorescence.

Complement C3↗

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↗

PubMed1998

The pathophysiological significance of nondesmoglein targets of pemphigus autoimmunity. Development of antibodies against keratinocyte cholinergic receptors in patients with pemphigus vulgaris and pemphigus foliaceus.

OBJECTIVES: To determine whether nondesmoglein (non-Dsg) autoantibodies are pathogenic and whether they recognize keratinocyte cholinergic receptors that control cell adhesion because antikeratinocyte autoimmunity in patients with pemphigus vulgaris is not limited to the development of autoantibodies to Dsg. DESIGN: To determine whether non-DSg autoantibodies are pathogenic, we sought to induce pemphigus in genetically engineered neonatal mice lacking Dsg 3 using pemphigus vulgaris IgGs that did not cross-react with Dsg 1. To determine whether pemphigus autoimmunity involves keratinocyte cholinergic receptors, the latter were separated from cell membranes of human keratinocytes, tagged with the covalent label [3H]propylbenzilylcholine mustard, and used as an antigen in a radioimmunoprecipitation assay of 34 pemphigus vulgaris and 6 pemphigus foliaceus serum samples. SETTING: The dermatologic clinics of the University of Minnesota, Minneapolis; the Mayo Clinic, Rochester, Minn; and the University of California-Davis Medical Center, Sacramento. PATIENTS: Serum samples were collected from 34 patients with pemphigus vulgaris and 6 patients with pemphigus foliaceus (aged 31-89 years) and from 7 age-similar patients of both sexes with nonpemphigus blistering or the following immune-mediated conditions: pemphigoid gestation, bullous drug eruption, lupus erythematosus, erythema nodosum, urticaria, acute contact dermatitis, and skin ulcers. MAIN OUTCOME MEASURES: Clinical, laboratory, and histopathologic findings. RESULTS: Extensive skin blistering accompanied by the Nikolsky sign and suprabasilar acantholysis was induced in the Dsg3null mice that received pemphigus, but not normal human IgGs. In the radioimmunoprecipitation assays for reactivity with cholinergic receptors, the mean radioactivity precipitated by pemphigus serum samples significantly exceeded both normal- and disease-control levels (P = .001-.02). The mean individual levels of radioactivity precipitated by 34 pemphigus vulgaris and pemphigus foliaceus serum samples (85%) exceeded control values by a mean of approximately 2.6 times. CONCLUSIONS: Autoantibodies to keratinocyte cell-surface molecules other than Dsg 1 and Dsg 3 can induce clinical features of pemphigus vulgaris. Patients with pemphigus vulgaris and those with pemphigus foliaceus develop IgG antibodies that precipitate radiolabeled cholinergic receptors. Because these receptors control keratinocyte adhesion and motility, their inactivation by autoantibodies may elicit intracellular signals that cause disassembly of desmosomes, leading to acantholysis and blistering.

Acantholysis↗

PubMed1990

Immunoblot analyses of Brazilian pemphigus foliaceus antigen using different antigen sources.

We investigated the Brazilian pemphigus foliaceus (BPf) antigen applying the immunoblotting method to two different antigen sources using 27 patients' sera. Twelve BPf sera reacted specifically with a 150 kD protein in extract of dispase separated human epidermis, while 18 sera yielded a similar protein band in bovine muzzle desmosomal preparation. The diversity of staining intensities between the two samples suggested the heterogeneity of BPf antigens in terms of epitopes. Japanese sporadic pemphigus foliaceus (Pf) sera showed similar results but Japanese pemphigus vulgaris (Pv) sera recognized different antigens of 130 kD or 135 kD, suggesting that BPf is similar to Japanese Pf but is distinct from Pv in respect to the antigenic substance. Furthermore, the present study showed that immunoblot analysis using different antigen sources should be a valuable tool to determine clinical types of pemphigus.

Animals↗

PubMed1999

Human desmocollin 1a transiently expressed in COS-7 cells and NIH 3T3-3 cells is reacted by IgG4 antibodies in a pemphigus foliaceus serum.

Human desmocollin (Dsc) 1 is an autoantigen in the subcorneal pustular dermatosis type of IgA pemphigus. Moreover, Dscs, particularly bovine Dscs, are recognized by IgG antibodies in certain sera of various types of pemphigus by immunoblotting. In this study, 10 pemphigus sera were examined by immunofluorescence for IgG antibodies to human Dsc1a, Dsc2a and Dsc3a transiently expressed in COS-7 and NIH 3T3-3 cells using two different transfection methods. IgG antibodies in a number of sera showed a strong background staining with untransfected COS-7 and NIH 3T3-3 cells. Using COS-7 cells, a pemphigus foliaceus serum, which did not stain untransfected COS-7 cells, was found to contain IgG antibodies reactive exclusively with Dsc1a. This serum showed the same reactivity in studies using NIH 3T3-3 cells. Moreover, this pemphigus foliaceus serum contained IgG4, but not IgG1 antibodies, to Dscla expressed in the NIH 3T3-3 cells. These results indicate that autoantibody response in pemphigus foliaceus might be more heterogenous than hitherto supposed.

3T3 Cells↗

PubMed2001

Thyroid gland tumour, pemphigus foliaceus and myasthenia gravis in the daughter of a woman with myasthenia gravis.

We describe a rare case of pemphigus foliaceus associated with familial myasthenia gravis (MG). A 35-year-old woman developed MG during oral corticosteroid treatment for pemphigus foliaceus. She had been operated on for a thyroid gland tumour that was confirmed histopathologically to be papillary carcinoma without metastasis. At the time of treatment, her mother had had MG for 30 years and undergone thymectomy 22 years ago. A specific ELISA technique showed that antidesmoglein 1 antibody was present in the daughter. There are many reports of multiple diseases such as pemphigus, thymoma, malignancy, and other autoimmune diseases associated with MG. However, familial MG following pemphigus foliaceus has not been reported previously.

Adult↗

PubMed1980

[Pemphigus foliaceus in a child. A two years follow-up under steroid. An immunosuppression therapy (author's transl)].

The observation of pemphigus foliaceus out of endemic zone in a six-year-old child is reported. The rate of anti-ICS antibodies is very high (1/6,400). The direct immunofluorescence shows the common aspect of epidermic network between the cells. Fluorescence basement membrane has been found with monospecific anti-C3 sera, and after remission with the monospecific anti-IgG sera. The clinical, histological and immunological relationship between pemphigus foliaceus in its sporadic and endemic forms and pemphigus erythematosus are discussed. The clinical and immunological regression is obtained by association of prednisone and immunodepressive therapy. After two years of treatment the maintenance dosis is not yet reached. A review of ten previous reported cases of pemphigus foliaceus in children in its sporadic form is presented.

Age Factors↗

PubMed1993

Ultrastructural localization of Brazilian pemphigus foliaceus (fogo selvagem) antigens in cultured human squamous cell carcinoma cells.

The ultrastructural localization of Brazilian pemphigus foliaceus (BPF) (fogo selvagem) antigen(s) in cultured human squamous cell carcinoma cells was studied using immunogold electron microscopy. Five of six BPF sera, which showed positive cell-surface reactivity on immunofluorescence, bound to the cell-cell contact area of cytoplasmic projections. This binding pattern was apparently different from that of non-endemic pemphigus foliaceus and pemphigus vulgaris sera, and mouse monoclonal anti-human E-cadherin antibody. The results suggest that BPF autoantibodies recognize a molecule(s) which is different from non-endemic pemphigus antigens, or different epitope(s) of a molecule identical with non-endemic pemphigus antigens, and that the epitope(s) to which BPF autoantibodies bind is expressed on cell-cell contact areas at a relatively early stage of cell-cell adhesion formation.

Autoantigens↗

PubMed1988

Complement and antibody deposition in Brazilian pemphigus foliaceus and correlation of disease activity with circulating antibodies.

Brazilian pemphigus foliaceus is a blistering skin disease endemic to central and southern areas of South America. In this study of skin biopsy specimens from 14 patients we present evidence that complement and immunoglobulins were present by direct immunofluorescence in the epidermal intercellular spaces in all patients. Eight of 14 patients had granular deposits of C3 in the basement membrane zone. By indirect immunofluorescence, serum samples from all 19 patients tested demonstrated the presence of circulating IgG autoantibody. Autoantibodies deposited in the intercellular spaces in titers ranging from 1:10 to more than 1:1280, and the titers drastically decreased during treatment. This is the first study to demonstrate complement deposition in the skin in Brazilian pemphigus foliaceus.

Adolescent↗

PubMed2002

Immunoablative high-dose cyclophosphamide without stem cell rescue in pemphigus foliaceus.

BACKGROUND: There is growing evidence that immunoablative high-dose cyclophosphamide without stem cell rescue is effective and safe in patients with refractory autoimmune diseases such as paraneoplastic pemphigus, systemic lupus erythematosus, aplastic anemia, and more recently pemphigus vulgaris. METHODS: We report a 51-year-old patient with severe pemphigus foliaceus, which was recalcitrant to multiple medical regimes. The patient presented with multiple thick hyperpigmented and scaly, ill-defined plaques on the face. In addition, she had multiple superficial erosions and crusts on her scalp, thorax, upper and lower extremities. The patient also had a few discrete intact flaccid bullae. A skin biopsy and direct immunofluorescence was consistent with pemphigus foliaceus. The patient's circulating pemphigus autoantibodies were present at a titer of 1 : 2560. The patient received immunoablative high-dose cyclophosphamide (50 mg/kg/day) for 4 consecutive days, and tolerated the regime well. RESULTS: Approximately 3 months after therapy, the skin lesions had healed and her prednisone, which had been as high as 80 mg daily, was tapered to 30 mg daily. In addition, her circulating autoantibodies decreased after treatment. Nearly 10 months after treatment, the patient did relapse. However, her disease was less severe and more easily managed with lower doses of immunosuppressive therapy. CONCLUSION: This case contributes to the growing evidence of high-dose cyclophosphamide's efficacy without stem cell rescue in recalcitrant autoimmune diseases, including pemphigus foliaceus.

Cyclophosphamide↗

PubMed1998

Pemphigus foliaceus: an unusual clinical feature and case reports.

Two cases of pemphigus foliaceus in 70-year-old women have been presented. The initial clinical picture, as well as results of histopathologic examinations, suggested the diagnosis of eczema. Because the therapy was not successful and changes in the clinical picture were observed, repeated histopathologic and immunofluorescent examinations were carried out. Presence of immunologic findings characteristic for pemphigus foliaceus formed the basis for verification of the diagnosis and administration of an appropriate therapy.

Aged↗

PubMed1995

Dapsone-induced motor peripheral neuropathy in pemphigus foliaceus.

We describe a case of pemphigus foliaceus complicated by dapsone-induced motor peripheral neuropathy. This idiosyncratic dapsone side-effect, which is rare other than in leprosy patients, was noteworthy for both its rapid onset, occurring within a month of commencing treatment, and its profound and prolonged nature.

Adult↗

PubMed1982

Coexisting childhood pemphigus foliaceus and Graves' disease.

We report herein the concurrent appearance of childhood pemphigus foliaceus and Graves' disease in a 14-year-old girl who was initially seen with crusted and hyperpigmented plaques on her chest, back, abdomen, and legs. The diagnosis of pemphigus foliaceus was confirmed by biopsy and immunofluorescent microscopic studies while the diagnosis of Graves' disease was based on clinical and laboratory findings. The coexistence of these two immune-mediated diseases in a single patient suggest that some persons may bear a unique predisposition to the development of two or more autoimmune-type disorders that may affect diverse organ systems.

Adolescent↗

PubMed2003

Pathogenicity and epitope characteristics of anti-desmoglein-1 from pemphigus foliaceus patients expressing only IgG1 autoantibodies.

Pemphigus foliaceus (PF) is an antibody-mediated autoimmune disorder with IgG1 and IgG4 as the predominant subclasses of autoantibodies against a desmosomal glycoprotein, desmoglein-1 (Dsg1). Previously, we found that the IgG4 anti-Dsg1 autoantibodies only recognize a conformational epitope(s), whereas the IgG1 autoantibodies recognize both conformational and linear epitopes but do not display pathogenicity in the passive transfer animal model. The purpose of this study was to analyze the epitopes recognized by autoanti-bodies from a subset of PF patients who only express anti-Dsg1 of the IgG1 isotype throughout the course of their diseases and to further characterize the pathogenicity of their IgG1 anti-Dsg1. We found that IgG1 auto-antibodies in this subset of PF patients, similar to IgG4 autoantibodies from other PF patients, are able to bind both human and mouse skin and induce the experimental PF in mice. Moreover, a detailed epitope mapping reveals that the conformational epitopes recognized by IgG1 autoantibodies from these PF patients are restricted to the first 161 amino acids of Dsg1, whereas the linear epitopes are spread throughout the entire ectodomain. In conclusion, our study reveals that the isotype of IgG does not necessarily determine the epitopes and pathogenicity of pemphigus autoantibodies.

Animals↗

PubMed2002

Intravenous immunoglobulin therapy for patients with pemphigus foliaceus unresponsive to conventional therapy.

BACKGROUND: Pemphigus foliaceus (PF) is a chronic autoimmune blistering skin disease that is commonly treated with oral corticosteroids and immunosuppressive therapy. In some patients, PF can be refractory to treatment and the resultant side effects of prolonged immune suppression can be potentially fatal. Alternative therapies are needed. OBJECTIVE: The purpose of this study is to report treatment outcomes with IVIg therapy in 11 patients with severe PF refractory to prednisone and other immunosuppressive therapy. METHODS: Selection criteria included documentation of a biopsy and immunopathology in 11 patients who were resistant to treatment or experienced side effects to conventional therapy. IVIg was administered according to a defined protocol. The parameters used to assess clinical response to IVIg included time observed for effective control of disease, duration of IVIg maintenance therapy, total duration of IVIg, number of IVIg cycles, systemic drug therapy, and the frequency of recurrences and relapses. The pre-IVIg and post-IVIg data were statistically analyzed by means of the SAS UNIVARIATE and 2-sided Wilcoxon sign rank and sign tests. RESULTS: All patients had an effective clinical response and remained in clinical remission for a mean period of 18.6 months after discontinuation of IVIg therapy. Serious side effects from IVIg use were not observed. CONCLUSION: IVIg therapy appears to have potential as a biologic alternative agent in inducing and maintaining clinical remissions in patients with PF who are resistant to more standard conventional treatment. IVIg is effective as monotherapy and may be needed for a period of several months to achieve a long-term clinical remission.

Adult↗

PubMed2003

Detection of mercury and other undetermined materials in skin biopsies of endemic pemphigus foliaceus.

A novel variant of endemic pemphigus foliaceus (EPF) was described among individuals in an area surrounding El Bagre, Colombia, South America. The population in this rural mining community is exposed to high environmental levels of mercury, used for gold extraction, as well as other minerals, metalloids, and trace elements (e.g., quartz, rutile, granite, magnetite, and almenite) and ultraviolet radiation. Fifty control subjects and fifty EPF patients in the endemic area were examined for the presence of mercury in skin biopsies and hair, using autometallographic and mass spectroscopic analyses, respectively. Simultaneously, serum levels of IgE were measured, and cutaneous tests for hypersensitivity reactions were performed. Using autometallography, mercuric sulfides/selenides were detected in 14 of 51 skin biopsies distributed similarly in the control and patient groups. However, significantly higher serum IgE levels and mercury concentrations in hair, urine, and nails were found in patients compared with controls. Microscopic analysis revealed mercuric sulfides/selenides concentrated within and around the sweat gland epithelium, as well as in dendritic cells. Five skin biopsies from EPF patients and five from controls that tested positive for the presence of mercuric sulfides/selenides by autometallography were randomly selected for electron microscopic analysis. This analysis revealed a mixed electron-dense and electron-light material closely associated with desmosomes in patients. However, there were intracellular vesicles containing an amalgam of electron-dense and electron-light materials only in the EPF patients. Thus, EPF-affected individuals are exposed to high levels of environmental mercuric sulfides/selenides and other elements. This is the first study reporting mercuric sulfides/selenides in skin biopsies from people living in a focus of EPF, and these compounds may play a role in the pathogenesis of autoimmunity.

Autoimmune Diseases↗

PubMed1993

Subclass reactivity of pemphigus foliaceus autoantibodies with recombinant human desmoglein.

Pemphigus foliaceus (PF) and its endemic form, Fogo Selvagem (FS), are autoimmune disorders characterized by subcorneal vesicles and IgG4 subclass autoantibodies that recognize a surface antigen of normal epidermal cells. FS and PF autoantibodies have been shown to bind desmoglein (DGI), a desmosomal glycoprotein classified as a member of the cadherin family of calcium-dependent cell adhesion molecules. In the present study we report the isolation of three overlapping cDNA clones representing greater than 90% of the extracellular domain of human DGI. Recombinant proteins encoded by these clones, designated DGI-1, DGI-2, and DGI-3, were produced in bacteria and analyzed for immunoblot (IB) reactivity with a panel of FS, PF, and control sera. FS and PF autoantibodies possessing reactivity with each of the three recombinant fusion proteins (FPs) were identified. FP DGI-3 (containing 123 amino acids of the membrane proximal region of the DGI ectodomain) showed reactivity with the largest number of patient sera--seven FS and one PF. IB reactivity with the DGI-1 FP (encoding 205 amino acids of the N-terminal region of DGI) could be eliminated by truncation of the C-terminal portion of this protein, indicating that autoantibodies were not binding the R-A-L motif. Autoantibodies reactive with two of the three FPs were predominantly restricted to IgG4, the subclass shown to be pathogenic in the passive transfer mouse model. The findings of this study demonstrate that the extracellular domain of DGI contains at least three antigenic sites recognized by FS and PF autoantibodies. The region near the membrane-spanning domain of DGI appears to contain an immunodominant site. This study is the first to document immunoblot reactivity of FS and PF autoantibodies with recombinant forms of DGI. The use of such molecular tools should facilitate the identification and characterization of relevant antigen/antibody systems in FS and PF.

Autoantibodies↗

PubMed2000

Pemphigus foliaceus successfully treated with mycophenolate mofetil as a steroid-sparing agent.

Pemphigus foliaceus is an autoimmune blistering disease of unknown origin with antibodies produced against desmoglein 1, an adhesive protein found in the desmosomal cell junction in the suprabasal layers of the epidermis. The disease is primarily treated with corticosteroids and corticosteroid-sparing immunosuppressive agents. We report a case of pemphigus foliaceus successfully treated with mycophenolate mofetil. It remains to be seen whether this agent has a significant effect on the course of the disease and remission induction.

Adult↗