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PubMed2004

Pemphigus foliaceus with neutrophilic spongiosis evolving to an atypical pemphigus phenotype.

A 46-year-old Brazilian man, with initial pustular lesions, neutrophilic spongiosis and subcorneal cleavage evolved to an atypical pemphigus phenotype, with suprabasal acantholysis. Interestingly, his autoantibody profile, tested by immunofluorescence, immunoblotting, enzyme-linked immunosorbent assay, and immunoprecipitation revealed exclusive IgG anti-desmoglein 1 antibodies in all phases of the disease.

Acantholysis↗
PubMed1989

Pemphigus foliaceus antigen: characterization of a keratinocyte envelope associated pool and preparation of a soluble immunoreactive fragment.

In both the endemic and sporadic forms of pemphigus foliaceus (PF), antiepidermal autoantibodies against desmoglein I are present. Desmoglein I is a highly insoluble 160-kD transmembrane glycoprotein of the desmosomal core. The detailed immunochemical characterization of the epitope(s) recognized by the PF autoantibodies is hampered by its large molecular weight and the insolubility of desmoglein I in nondenaturing buffers. This study was designed to identify alternative methods that could yield soluble immunoreactive PF antigen (Ag) from normal human epidermis. The presence of PF Ag in human epidermis and in its soluble or insoluble fractions was monitored by indirect immunofluorescence, immunoadsorption of PF sera, and immunoprecipitation of radiolabeled fractions. The PF Ag from trypsin-resistant, radiolabeled cell envelope preparations was cleaved by papain and immunoprecipitated by PF sera. A 50-kD peptide, isoelectric at pH 5.5-5.8, was immunoprecipitated by sera from all patients with endemic PF (n = 15) or idiopathic PF (n = 4), and by two of four pemphigus vulgaris sera, but by no control sera (n = 7). This study shows that a significant fraction of the PF Ag is insoluble, trypsin-resistant, and is associated with the cornified cell envelope fraction, but an Ag fragment can be obtained in a small molecular weight, soluble, and immunoreactive form by papain digestion. This 50-kD papain fragment is more amenable to detailed chemical and immunologic characterization than the native molecule.

Antigens↗
PubMed2002

Tunisian endemic pemphigus foliaceus is associated with desmoglein 1 gene polymorphism.

Desmoglein 1 is the target antigen and probably the initiating immunogen of the autoantibody response in pemphigus foliaceus (PF), a blistering autoimmune skin disease. We previously showed that the desmoglein 1 gene (DSG1) is polymorphic and that one of its variants is associated with the sporadic form of PF observed in France. Herewith, we report, based on a case-control analysis, that the same DSG1 polymorphism participates in susceptibility to the endemic form of PF seen in Tunisia and, thus, show that common genetic factors govern the breakage of tolerance to desmoglein 1 in different epidemiological and environmental situations.

Adolescent↗
PubMed1990

Detection of pemphigus vulgaris and pemphigus foliaceus antigens by immunoblot analysis using different antigen sources.

In an immunoblot analysis with human epidermal extract as a source of antigens, all (28/28) pemphigus vulgaris (Pv) sera showed a specific reactivity with a 130-kD protein. Several, but not all, Pv sera reacted with similar antigens in both a bovine muzzle desmosome preparation and extract of cultured human squamous carcinoma cells. On the other hand, some pemphigus foliaceus (Pf) sera exhibited reactivity with a 150-kD protein, which is most likely desmoglein I, in both the human epidermal extract and the bovine desmosome preparation, but no Pf serum reacted with this antigen in the squamous carcinoma cell extract. Furthermore, 4/16 Pv sera also reacted with a 150-kD protein in the desmosome preparation, which seemed to be the same as Pf antigen. These results show a relationship between antigens of both Pf and Pv and desmosomes, as well as heterogeneities of both Pv and Pf antigens in terms of antigenic molecules or epitopes. Furthermore, this study presents the possibility that immunoblot analysis can be routinely used for differentiation of Pv and Pf antibodies.

Antigens↗
PubMed1990

IgA pemphigus foliaceus: a case report.

An 84-year-old male with a 4-week history of vesiculobullous eruptions was diagnosed clinically and pathologically to suffer from pemphigus foliaceus. Amazingly, direct immunofluorescence demonstrated intercellular IgA deposits in the whole epidermis. The patient did not respond to a regimen of fluocortolone and azathioprine but rapidly improved under dapsone. The few reported dermatoses with intraepidermal IgA deposits do not form a homogeneous group. In the following, their spectrum will be outlined. It is important to distinguish these dermatoses from IgG pemphigus, since they require a different therapy.

Aged↗
PubMed1980

South American pemphigus foliaceus: electron microscopy and immunoelectron localization of bound immunoglobulin in the skin and oral mucosa.

Five skin and two oral biopsies from patients with South American pemphigus foliaceus (SAPF) were studied by electron and immunoelectron microscopy for the ultrastructural localization of bound immunoglobulin in epidermal and oral lesions. Electron microscopy showed the tonofilament-desmosome complex to be preserved in the various layers of the epidermis. Immunoglobulin was bound over the plasma membrane and permeated the desmosomal junctions both in the skin and oral mucosa, thus suggesting that pemphigus antibodies are attached to the glycocalyx. It appears that the initial injury in SAPF acantholysis involves the glycocalyx and that it might be caused by interaction with intercellular antibodies present in the patient's serum.

Adult↗
PubMed1995

Pemphigus vulgaris and pemphigus foliaceus sera show an inversely graded binding pattern to extracellular regions of desmosomes in different layers of human epidermis.

We analyzed the location of binding sites for pemphigus vulgaris (PV) antigen and pemphigus foliaceus (PF) antigen in the human epidermis using serum samples obtained from three patients with PV and three patients with PF. Confocal laser scanning microscopy, immunofluorescent examination of ultrathin cryosections, and immunoperoxidase electron microscopy demonstrated discontinuous dots along the epidermal cell surfaces. Immunogold electron microscopy of ultrathin cryosections showed specific binding of PV and PF autoantibodies only to desmosomes. Post-embedding immunogold electron microscopy using cryofixation and cryosubstitution enabled the whole depth of the epidermis to be examined and the binding of PV and PF autoantibodies to be quantitated by counting gold particles. Both PV and PF autoantibodies bound to all desmosomes in the epidermis, but not to the surface of the non-desmosomal keratinocytes. The majority of auto-antibody binding occurred in the extracellular domain (PV, 62%; PF, 69%). The statistical analysis of two-way analysis of variance regarding the number of gold particles labeling a single desmosome confirmed a significant interaction between subtypes of pemphigus (PV and PF) and the different epidermal cell layers (p < 0.044). The results indicate that the number of gold particles bound to individual desmosomes with PV sera was significantly higher in the lower epidermis than in the upper epidermis, and that of PF sera showed reciprocal pattern. This inversely graded binding pattern suggests heterogeneity of the composition of the desmosomes, which may explain the differences in level of acantholysis between PV and PF.

Autoantibodies↗
PubMed1982

Use of a gold compound for the treatment of pemphigus foliaceus in a foal.

A 4-month-old foal was examined because of generalized exfoliative dermatitis, with thick scales, extensive crusting, and oozing of serum. A diagnosis of pemphigus foliaceus was made by histologic and immunopathologic examinations of skin biopsy specimens. The foal was treated with a gold compound, aurothioglucose (1 mg/kg once weekly for 14 weeks). For the initial 5 weeks, high-dose glucocorticoid treatments also was used. The skin disorder resolved entirely after 12 weeks of treatment.

Animals↗
PubMed2005

[A case of sporadic pemphigus foliaceus in teenage girl with psoriasis vulgaris].

A 15-year-old girl with generalized erythematous, scaling plaques, bullous and erosive lesions on the skin was diagnosed as having pemphigus foliaceus. These symptoms occurred during the topical treatment for psoriasis vulgaris. Confirmation of diagnosis was made by routine histopathology and both direct and indirect immunofluorescence microscopy. The patient's serum gave positive reaction against desmoglein 1 in ELISA technique. The patient was successfully treated with systemic corticosteroids (at the beginning intravenous methylprednisolone) and immunosuppressants (cyclophosphamide p.o.).

Adolescent↗
PubMed1986

Complement fixation by Brazilian pemphigus foliaceus antibodies in vitro.

In order to examine the presence of intercellular substance antibodies (ICS Ab) which will fix complement in vitro, in the sera of patients with Brazilian pemphigus foliaceus (BPF), 7 BPF sera were tested using immunofluorescence (IF) and complement IF (CIF). All 7 sera possessed IgG ICS Ab at titers varying from 1/320 to 1/2,560 (normal human skin as substrates). Using CIF, 2 out of 7 sera proved to have a capacity to fix complement (C3) in vitro at a titer of 1/40. Although the titer of complement-fixing ICS Ab detected by CIF was lower than IgG ICS Ab detected by IF, these results with the evidence of frequent C3 deposition in the lesional skin of BPF suggest the necessity of further studies on the role of complement system in BPF.

Autoantibodies↗
PubMed1998

[Adjuvant therapy of pemphigus vulgaris and pemphigus foliaceus with intravenous immunoglobulins].

High dose intravenous immunoglobulins (IVIG) have been shown to be effective in different autoimmune diseases. We report on the use of IVIG in patients with pemphigus vulgaris (PV) or pemphigus foliaceus (PF) resistant to conventional therapy. Six patients who presented with a relapse of their disease following conventional immunosuppressive treatment with prednisolone and azathioprine were additionally treated with 2 g/kg of IVIG every four weeks. Six to nine cycles of this therapy were given to each patient. All patients showed healing of their blisters without evidence of relapse even when steroids and immunosuppressants were tapered. None of the patients relapsed within one year of follow-up, although IVIG treatment was discontinued. Side effects of IVIG treatment were moderate with only slight evidence of headaches. We suggest that adjuvant treatment of PV and PF resistant to conventional immunosuppressive strategies is a useful addition to our therapeutic arsenal in both diseases.

Autoantibodies↗
PubMed2005

A canine pemphigus foliaceus case showing parallel relationship of disease activity and titer of serum anti-keratinocyte cell surface antibodies.

A seven-year-old, spayed female mongrel dog was diagnosed as pemphigus foliaceus (PF) by clinical, histopathological and immunopathological observations. Serum antibodies against the cell surface of keratinocytes in the dog were detected by indirect immunofluorescence (IIF) using cryosectioned bovine esophagus as well as living cultured-canine keratinocytes as the substrates. When we compared the titers of IIF on bovine esophagus with its disease activity, the IIF titers reflected the disease activity throughout the time course. Our findings will suggest that sequential titration of serum antibodies by IIF will be useful for monitoring the serological disease activity in canine PF.

Animals↗
PubMed2000

Thymoma with pemphigus foliaceus.

A 75-year-old Japanese woman was referred to us because of an anterior mediastinal mass. Crusts and shallow erosions developed 10 months earlier on her upper chest, back, and scalp. Pemphigus foliaceus was diagnosed based on histological examination of skin biopsy specimens and positivity for serum anti-desmoglein 1 antibody by enzyme-linked immunosorbent assay. Neurological examination and electromyography ruled out myasthenia gravis. Total thymectomy was performed, and the postoperative pathology studies showed mixed lymphoepithelial thymoma. One year after the resection, the eruption and alopecia improved and the serum anti-desmoglein 1 antibody titer decreased, suggesting a beneficial effect of thymectomy on thymoma-related pemphigus.

Aged↗
PubMed1988

Pemphigus foliaceus with anti-intercellular and anti-basement membrane zone antibodies. Blocking immunofluorescence and Western immunoblotting studies.

In a patient with clinical, histologic, and routine direct immunofluorescence (IF) findings compatible with pemphigus foliaceus (PF), anti-intercellular and anti-basement membrane zone (BMZ) antibodies were found on indirect IF. Blocking IF studies using the biotin-avidin method revealed that intercellular staining of this patient's serum was significantly decreased by sera of PF patients, however, reaction of this patient's serum was not blocked by sera of PV and BP patients. Western immunoblot analysis using the SDS extracts of normal human epidermis demonstrated that this patient's serum reacted with 370-, 220-, 170-, 130- and 21-kD proteins. Another PF serum reacted with 170- and 21-kD proteins. BP sera reacted with 220-, 68-, 46 and 21-kD proteins. Considering the results of the previous reports and this study, 220- and 170-kD protein bands are specific for BP and PF, respectively, and this is the first case of PF with both anti-intercellular and anti-BMZ circulating antibodies including immunoblotting analysis.

Aged↗
PubMed1991

Childhood pemphigus foliaceus.

We describe a girl who presented at the age of 12 years with a blistering eruption on the lower limbs. One week later, numerous new bullae and blisters were present. Skin biopsy for histology and immunofluorescent studies established the diagnosis of pemphigus foliaceus.

Azathioprine↗
PubMed2005

[Pemphigus foliaceus in a Haflinger gelding].

A 10-year-old Haflinger gelding was presented with severe generalized chronic dermatitis characterized by scales, crusts and widespread alopecia with a partially diffuse and partially circumscribed pattern. Pemphigus foliaceus was diagnosed based on history, clinical signs and histological examination of skin biopsies. Typical histological findings were subcorneal pustules with accumulations of intact neutrophil granulocytes and acantholytic keratinocytes. The gelding was treated with glucocorticoids and gold salts. The skin lesions resolved completely after 8 weeks of treatment. No recurrence was observed within 1 year.

Animals↗
PubMed2005

Pemphigus foliaceus and desmoglein 1 gene polymorphism: is there any relationship?

Transmembrane proteins of the cadherin superfamily, the desmogleins and desmocollins, mediate intercellular adhesion in desmosomes. Autoantibodies to desmoglein 1 (dsg1) are a hallmark of pemphigus foliaceus (PF), a disease characterized by skin blistering resulting from keratinocyte cell detachment. The etiology and pathogenesis of this disease remain poorly understood; however, genetic susceptibility is clearly involved. The aim of this study was to verify if genetic variants of dsg1 influence susceptibility/resistance to endemic PF (fogo selvagem). Two single nucleotide polymorphisms (SNPs) were analyzed: 809 (C,T), a synonymous variation, and 1660 (A,C), a tyrosine<-->serine variation in the fifth extracellular domain. Allelic, haplotypic and genotypic frequencies did not differ significantly between the patient (n=134) and the control (n=227) population samples. Moreover, there is no evidence of interaction between the DSG1 and the HLA-DRB1 and IL6 genes, whose alleles had been found associated with differential susceptibility to PF. The results of this study agree with the described and predicted B- and T-cell epitopes of the dsg1 molecule, which seemingly are not affected by the allelic variation. We conclude that genetic diversity of the autoantigen dsg1 is not a major factor for PF pathogenesis in the Brazilian population.

Amino Acid Substitution↗