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PubMed2006

Atypical pemphigus: discordance between clinicopathological findings and the antigenic profile in four cases.

BACKGROUND: The diagnosis of pemphigus vulgaris and pemphigus foliaceus is usually based on clinical, histological, and immunofluorescence (IF) findings. In recent years, the antigenic profile of both diseases has been further defined by immunobiochemical techniques (ELISA, immunoblot, and immunoprecipitation). METHODS: A retrospective study of 40 pemphigus patients was performed to determine the clinical, histological, and antigenic profile in patients with pemphigus followed at our Department. Charts review, clinical data, histological and IF findings, and antigenic analysis by ELISA were performed in all patients. RESULTS: In most patients, there was a perfect correlation between the clinical and histological findings and their antigenic profile. In four patients (10%), clinicopathological features and antigenic findings were discordant. CONCLUSIONS: The antigenic profiles in pemphigus do not always correlate with the clinical diagnosis. Therefore, clinical and histological features should be considered as the mainstay for the diagnosis of pemphigus.

Aged↗
PubMed2001

Immunoreactivity against intracellular domains of desmogleins in pemphigus.

In pemphigus vulgaris (PV) and pemphigus foliaceus (PF), most of the autoantibodies are directed against the extracellular domains of desmoglein 1 (Dsg1) or Dsg3, and those antibodies are proved to play a pathogenic role in blister formation in the skin and mucous membranes. However, some pemphigus sera have been reported to react with the intracellular domains of these antigens. In the present study, we examined the reactivity of the sera from various types of pemphigus with recombinant proteins of extracellular and intracellular domains of human Dsg1 and Dsg3 by immunoblot analysis. We produced the entire extracellular domain of Dsg1 or Dsg3 fused with mouse IgG2a by baculovirus expression. We prepared the intracellular domain of Dsg1 or Dsg3 fused with glutathione-S-transferase by bacterial expression. All of the 31 PV sera reacted with the extracellular domain of Dsg3 and four reacted with the intracellular domain. Six out of 19 PF sera reacted with the extracellular domain of Dsg1 and five reacted with the intracellular domain. In addition, some sera of Brazilian PF patients or cases with mixed features of PV and PF also reacted with the intracellular domains of Dsg1 or Dsg3. Although the frequency was low, some sera did react with the intracellular domain of Dsgs.

Baculoviridae↗
PubMed2000

The use of two substrates to improve the sensitivity of indirect immunofluorescence in the diagnosis of pemphigus.

The aim of this study was to re-evaluate indirect immunofluorescence (IIF) comparing two substrates, normal human skin (HS) and monkey oesophagus (MO) using serum from 29 pemphigus patients classified according to the presence of serum autoantibodies to either desmoglein (Dsg) 1 or Dsg3 detected by enzyme-linked immunosorbent assay (ELISA). Overall, the sensitivity of IIF was 83% on HS and 90% on MO. When data from both substrates were combined, the sensitivity increased to 100%. When sera from pemphigus foliaceus (PF) patients were studied, which contained Dsg1 antibodies only, the sensitivity of IIF was greatest on HS and titres were on average 4.8 doubling dilutions higher than on MO. In contrast, when sera containing autoantibodies only to Dsg3 from pemphigus vulgaris (PV) patients was studied, the sensitivity was greatest on MO and titres were on average 4.4 doubling dilutions higher than on HS. There was a significant correlation between Dsg1 antibody levels and IIF titres on HS and between Dsg3 antibody levels and IIF titres on MO. The investigation of immunobullous disorders in the future is likely to move towards antigen-specific techniques such as the Dsg ELISAs used in this study. However, in laboratories which currently rely on IIF for detecting pemphigus autoantibodies, the data presented in this study strongly suggest that two substrates should be used for IIF screening: one rich in Dsg1, such as HS, and the other rich in Dsg3, such as MO. This combination of substrates should not only increase the sensitivity of detecting pemphigus antibodies, but will aid in the differentiation of PV from PF. It is also possible that the data might be more useful for disease monitoring.

Animals↗
PubMed1976

[Herpetiforme pemphigus].

The case is presented of a woman, who for seven years had vesicobullous outbreaks, typical of Dermatitis herpetiformis, with an extraordinary itching. The first biopsy had a doub acantholysis but the following one showed: "eccematoid-like spongiosis with eosinophilia" and "subepidermal bulla with eosinophilia, erythema multiformislike". The evolution of the case was to pemphigus foliaceus, and by the action of long-standing corthicotherapy ended in a typical Senear-Usher with present good health. These anomalous cases, first described clinically by Brocq, and histologically by Unna et Leredde, are best known now as "Pemphigus herpetiformis" (Jablonska et al.).

Biopsy↗
PubMed1994

Thiopronine-induced herpetiform pemphigus: report of a case studied by immunoelectron microscopy and immunoblot analysis.

We report a case of herpetiform pemphigus induced by thiopronine. Direct immunoelectron microscopy performed on peribullous skin showed IgG deposits predominantly located in the extracellular portion of desmosomes. By immunoblot analysis using bovine tongue extracts as the antigen, the patient's serum recognized a 160-kDa polypeptide which comigrated with desmoglein I. This study underlines the contribution of immunoblot analysis to the diagnosis of atypical cases of pemphigus, and confirms that herpetiform pemphigus may be a clinical variant of pemphigus foliaceus.

Aged↗
PubMed2001

Further support for a role for Th2-like cytokines in blister formation of pemphigus.

Pemphigus vulgaris and pemphigus foliaceus are commonly known as antibody-mediated bullous diseases. However, recently a role for infiltrating cells as contributors to the pathogenesis of these diseases has been suggested. The aims of our study were to characterize the immunophenotype of the cellular infiltrate of pemphigus lesional skin and to study the cytokines secreted. We have therefore performed an immunohistochemical study with a large panel of monoclonal antibodies (to CD3, CD4, CD8, CD25, CD30, myeloperoxidase, eosinophil cationic protein EG2, tryptase, human interleukin (IL)-2, human IL-4, human IL-5, human IL-6, human IL-8, and interferon (IFN)-gamma using the alkaline phosphatase-antialkaline phosphatase procedure on lesional and uninvolved skin of six patients with clinical, histological, and immunofluorescent proven pemphigus. We also performed RT-PCR in order to demonstrate mRNA expression of the cytokines of interest. Our results suggest the presence of a T cell population with a prevalent Th2-like cytokine pattern in lesional skin. In addition, we demonstrate a consistent number of granulocytes and mast cells that show clear signs of activation. These data suggest the involvement of an inflammatory infiltrate in the production of pemphigus lesions. In particular, we assume that Th2 cells may be implicated in the very early stages of autoimmune response, concluding that they exert broad activity in blister formation.

Adult↗
PubMed2000

Drug-related pemphigus and angiotensin converting enzyme inhibitors.

A 105-year-old woman developed pemphigus foliaceus. She had been on fosinopril, an angiotensin-converting enzyme inhibitor (ACE inhibitor) for 4 years. Anti-intercellular cement substance antibodies were positive with titre > 160. She died during admission of an unrelated illness. A 57-year-old man developed pemphigus vulgaris after 11 months treatment with quinapril. At 14 months after developing pemphigus, this man continues on prednisone and azathioprine. We speculate that these are cases of ACE-inhibitor-related pemphigus and we review ACE-inhibitor-related pemphigus.

Aged↗
PubMed1990

Pemphigus immunoglobulin G subclass autoantibodies: studies of reactivity with cultured human keratinocytes.

The subclass distribution of pemphigus vulgaris (PV) autoantibodies has been investigated further. Cultured human keratinocytes (HuKs) were incubated with immunoglobulin G (IgG) fractions prepared from the serum samples of five patients with PV and one patient with pemphigus foliaceus (PF). The binding of IgG subclasses to HuK was detected by the use of indirect immunofluorescence done with monoclonal antibodies that were specific for each of the four human IgG subclasses. IgG1 and IgG4 binding to keratinocytes was detected in all of the pemphigus IgG preparations. In each instance, the titer of bound IgG1 was equal to or greater than the IgG4 titer. IgG3 binding was detected in only one PV IgG, and IgG2 pemphigus antibody activity was not detected. Differences in the immunofluorescence patterns between the five PV and one PF IgG and between the five PV and a second PF serum were noted. PV antigens were detected as a speckled pattern on HuKs, with accentuation in the intercellular spaces. By comparison, the pattern of PF antigen expression varied significantly depending on whether anti-IgG1 or anti-IgG4 antisera were used. Anti-IgG1 bound in a coarse granular pattern without accentuation in the intercellular spaces, but anti-IgG4 bound in a pattern nearly identical to that observed with PV IgG and its subclasses.

Antibodies, Monoclonal↗
PubMed1979

Immunopathologic and histologic studies on skin from pemphigus patients in tissue culture.

Explants of skin from patients with pemphigus vulgaris and pemphigus foliaceus taken in the active stages of the disease had in vivo bound IgG in the intercellular area. After 24-48 h incubation of these explants in normal sera acantholytic bullae developed above the stratum basale, thus indicating that the bound IgG is probably in vivo bound pemphigus antibody. In both cases, skin from the inactive stage of the disease contained no in vivo bound pemphigus antibodies. Explants of these skin specimens failed to develop acantholysis in culture.

Acantholysis↗
PubMed1998

HLA class II allele associations in Korean patients with pemphigus.

BACKGROUND: Studies at the population level have demonstrated associations between pemphigus (pemphigus vulgaris, PV, and pemphigus foliaceus, PF) and particular HLA haplotypes, which suggests that there may be a genetic predisposition for the disease. OBJECTIVE/METHODS: The aim of the present study was to examine the distribution/frequency pattern of HLA class II alleles (DRB1, DQA1 and DQB1) from a group of 30 Korean patients with pemphigus (15 PV and 15 PF) by PCR amplification with sequence-specific primers. RESULTS: In PV, the frequency of DRB1*01 allele was found to be significantly high (pc = 0.0014); in PF, DRB1*01, DQA1*0302 and DQB1*0603 alleles showed positive associations with statistical significances (pc = 0.0002, 0.0007 and 0.0067, respectively), when compared with those found in Korean controls. CONCLUSIONS: In this small-sample study, findings of allelic frequencies among Korean patients with pemphigus are somewhat different from those found in other populations.

Alleles↗
PubMed1980

Binding of bullous pemphigoid antibodies to basal cells.

Eight out of 12 serum samples from patients with bullous pemphigoid having basement membrane zone antibodies gave positive binding not only to the basement membrane zone but also to the basal cell membrane and/or cytoplasm as observed by complement immunofluorescence. Reaction of fluorescein labeled pemphigus vulgaris gamma-globulins, binding mainly to the lower intercellular spaces of the epidermis, was greatly reduced by the prior incubation of high-titered bullous pemphigoid sera having the reactivity to the basal cells, while that of fluorescein labeled pemphigus foliaceus gamma-globulins binding mainly to the upper and middle intercellular spaces, was not influenced by the prior application of these bullous pemphigoid sera. These results indicate that bullous pemphigoid antibodies are heterogenous and can be classified into 2 types and that some cross reaction is present between pemphigus antibodies and bullous pemphigoid antibodies having the reactivity to the basal cells.

Antigen-Antibody Reactions↗
PubMed2006

Prolonged clinical remission of patients with severe pemphigus upon rapid removal of desmoglein-reactive autoantibodies by immunoadsorption.

BACKGROUND: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus foliaceus (PF), respectively. Therapeutic removal of Dsg-reactive autoantibodies by immunoadsorption (IA) has been demonstrated to exert clinical remission of the disease. OBJECTIVES: The aim of this intervention study was to evaluate the efficacy and safety of the peptide-based Globaffin adsorber system in the treatment of severe pemphigus cases. PATIENTS AND METHODS: We applied IA in 4 PV and 2 PF patients with severe chronic disease resistant to conventional immunosuppressive therapy. IA was performed on 4 consecutive days, representing 1 treatment cycle, followed by a 4-week treatment-free interval. Serum samples for determining serum IgG and anti-Dsg1/Dsg3 IgG autoantibodies were drawn daily before and after IA, respectively. During follow-up, patients were examined carefully, and laboratory parameters were controlled monthly for up to 1 year. RESULTS: IA led to excellent clinical responses. Skin and mucosal lesions cleared almost completely within weeks. One IA cycle reduced anti-Dsg1 and anti-Dsg3 autoantibodies by an average of 50-70% as determined by ELISA. CONCLUSIONS: Using the Globaffin adsorber system, IA represents an effective and safe treatment opportunity in severe and therapy-resistant pemphigus.

Adult↗
PubMed1994

Atypical pemphigus with concomitant IgG and IgA anti-intercellular autoantibodies associated with monoclonal IgA gammopathy.

A case is reported of a 60-year-old woman with acantholytic vesiculopustular dermatosis and IgA-lambda monoclonal gammopathy. The histopathology of vesiculopustular lesions showed intraepidermal acantholytic and neutrophilic blisters. Direct immunofluorescence revealed intercellular (IC) IgD deposition with concurrent deposits of IgA and C3. Indirect immunofluorescence and immunoblotting studies revealed that the patient had circulating IgG and IgA anti-IC antibodies both of which recognized the 150-kD desmoglein that was pemphigus foliaceus antigen in a bovine desmosome preparation.

Autoantibodies↗
PubMed2006

Proteomic analysis of the autoantibody response following immunization with a single autoantigen.

In most autoimmune diseases, the autoantibody response is directed against several antigens of the target organ whose identification is crucial for understanding the physiopathological process. Thus, technologies allowing a characterization of the whole autoantibody pattern of both human and experimental autoimmune diseases are required. Here we have used immunoproteomic analysis of human epidermal extracts to characterize the diversity of the anti-desmosome antibody response induced in normal mice immunized with desmoglein 1, the major autoantigen of pemphigus foliaceus, an autoimmune blistering skin disease. In particular, this analysis enables us to characterize the binding properties of anti-desmosome mAbs derived from these mice and to show that the autoantibody response induced upon immunization with a single autoantigen targets different epidermal autoantigens with a pattern similar to that observed in certain variety of human pemphigus.

Amino Acid Sequence↗
PubMed1988

Pemphigus-like eruption induced by d-penicillamine and captopril, in the same patient.

A patient suffering from rheumatoid arthritis and hypertension is described who developed pemphigus foliaceus during simultaneous treatment with d-penicillamine and captopril. Discontinuation of the d-penicillamine afforded some improvement, but skin symptoms still persisted after 3 months. When the captopril was discontinued as well, all lesions healed within 2 weeks. Our findings suggest that both these chemically related drugs might have been responsible for the pemphigus-like eruption in this patient.

Captopril↗
PubMed2000

Detection of human herpesvirus 8 DNA in pemphigus and chronic blistering skin diseases.

Increased incidences of Kaposi's sarcoma and lymphoid malignancies have been observed in patients with pemphigus, and the human herpesvirus 8 (HHV-8) is very strongly associated with these tumors. Because the virus may be one of the triggering factors of pemphigus, we undertook this study to screen for the presence of HHV-8 in chronic blistering skin diseases including pemphigus. A total of 45 paraffin-embedded specimens were studied using nested polymerase chain reaction (PCR) with primers to amplify a 160-base pair HHV-8 fragment. HHV-8 DNA could be detected in 7 of 9 patients with pemphigus vulagris, and 1 of 2 with pemphigus foliaceus. All specimens of other blistering skin diseases were negative for HHV-8. On sequencing PCR products, the sequences were almost identical with the prototypic sequence for HHV-8, and a few base- pair substitutions at 1086C-T and 1139A-C were detected. The results of our study suggests that HHV-8 might have trophism for pemphigus lesions. Further studies including comparison of HHV-8 DNA load in both lesional and normal skin in the same patient, serological and animal studies would be helpful to study the relationship between HHV-8 and pemphigus.

Adult↗
PubMed1996

Pemphigus: from immunofluorescence to molecular biology.

Since the discovery of autoantibodies in patients with pemphigus, pemphigus has been intensively studied by dermatologists and cutaneous or cellular biologists by means of various techniques including immunofluorescence, immunoelectron microscopy, immunoprecipitation, immunoblotting, and molecular biology. In this article, up-dated topics on pemphigus obtained by each individual technique are reviewed. In the course of immunofluorescence studies on unusual cases of blistering diseases, a new entity characterized by immunoglobulin A (IgA)-type autoantibodies directed against keratinocyte cell surfaces has been discovered. Immunoelectron microscopy using low temperature post-embedding gold labeling enabled us to quantitate binding sites of pemphigus autoantibodies within desmosomes at different levels of epidermis. Immunoprecipitation and immunoblot analyses allowed us to characterize antigen complexes in paraneoplastic pemphigus. Finally, approaches using molecular biology not only have given us a fundamental insight that pemphigus autoantigen is a cadherin-type cell adhesion molecule both in pemphigus vulgaris and pemphigus foliaceus, but also provided tools to develop novel diagnostic and therapeutic strategies.

Autoantibodies↗
PubMed1982

Induced pemphigus.

The reports of induced pemphigus have multiplied over the last 10 years. Several heterogeneous factors, such as drugs (penicillamine, pyritinol, captopril, rifampicin, etc.), physical agents (burns, UV, and ionizing radiation) and viruses, can play an inducing role. Usually, the disease is preceded by prodromal, non-specific lesions. The full-blown stage shows features of pemphigus foliaceus, erythematosus or herpetiformis. Histologically, acantholytic splits mostly occur at the highest malpighian layers. Intercellular antibodies and frequently concomitant other auto-antibodies are found in the serum, the titre of the former usually being low and unrelated to the severity or course of the disease. The biological progress is variable and ranges from rapidly and definitively healing cases (induced pemphigus proper) to others which, in spite of the elimination of the inducing factor, self perpetuate just like true pemphigus (triggered pemphigus). Pathogenic hypotheses are based on the act that some inducing factors can alter the antigen distribution on keratinocyte membranes and/or interfere with immune surveillance by impairing T-suppressor cells.

Antibodies↗