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PubMed2003

Human placental amnion is a novel substrate for detecting autoantibodies in autoimmune bullous diseases by immunoblotting.

BACKGROUND: Identification of antigens by immunoblotting techniques, using epidermal and dermal extracts, is regarded as essential for making a definitive diagnosis in autoimmune bullous diseases (AIBDs). These procedures involve epidermal-dermal separation for subsequent protein extraction, which may result in partial loss of some antigenic polypeptides and changes in the conformational epitopes targeted by autoantibodies in AIBDs. It may therefore be necessary to use different substrates for consistent results. Objectives To evaluate the usefulness of human placental amnion extract as a substrate for immunoblotting in the diagnosis of AIBDs. METHODS: We checked the structural components of the desmosomes and basement membrane zone (BMZ) of amnion by electron microscopy. Using immunofluorescence and immunoblotting techniques, we tested the amnion immunoreactivity with antibodies to desmosomal and BMZ proteins, and with sera from 76 patients with AIBDs including pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid (BP), pemphigoid gestationis, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, paraneoplastic pemphigus and mucous membrane pemphigoid. RESULTS: The desmosomes and BMZ of the amnion tissue were ultrastructurally similar to those in skin. Antigen mapping confirmed that amnion contains all the proteins that were recognized by a panel of monoclonal and polyclonal antibodies. Immunoblotting showed that the antibodies clearly detected bands corresponding to desmogleins 1 and 3, desmocollins 1 and 2, desmoplakins 1 and 2, three subunits (alpha3, beta3 and gamma2) of laminin 5, BP antigens 1 and 2, the 97-kDa LAD antigen and type VII collagen. In addition, most of the patient sera (82%) reacted exclusively with their respective antigens. CONCLUSIONS: Harvesting proteins from amnion does not require epidermal-dermal separation, and a sufficient yield of desmosomal and hemidesmosomal proteins can be obtained. Therefore, amnion may be a more reliable source of substrate than skin samples for immunoblot analysis of AIBDs.

Amnion↗
PubMed2005

Pemphigus: analysis of 1209 cases.

BACKGROUND: Pemphigus is a rare and chronic life-threatening disease. The clinical picture varies in reports from different regions of the world. OBJECTIVE: To define the clinical forms of pemphigus in a large cohort of patients. METHODS: Prospective analysis of 1209 patients diagnosed and followed at the Pemphigus Research Unit, Tehran University for Medical Sciences, from 1984 to 2003. RESULTS: The mean age at onset was 42 years with a female to male ratio of 1.5/1. The most frequent form was pemphigus vulgaris. In pemphigus vulgaris, patients' mucous membrane involvement alone was observed in 18%, skin involvement alone in 12%, and both in 70%. Pemphigus foliaceus was observed in 7% of the patients. Most complications were iatrogenic. CONCLUSION: In Iran, pemphigus vulgaris is the most frequent form of pemphigus. Females are more prone to the disease. The incidence of pemphigus in Tehran is approximately 1.6 per 100,000/year, and in Iran 1.0 per 100,000/year. The age of onset was lower than classically reported. Death occurred in 6.2% of the patients. In pemphigus vulgaris, the mucosal and skin form together had a worse prognosis than the other clinical forms.

Adolescent↗
PubMed1989

Pemphigus and Kaposi's sarcoma.

A 77-year-old woman developed Kaposi's sarcoma (KS) during prednisone therapy for pemphigus foliaceus. There are 7 prior reports in the literature of KS occurring in association with pemphigus. There are multiple reports of KS occurring in patients without pemphigus who are immunosuppressed either by malignancy or by chemotherapy for various disorders. Although the relationship between KS and pemphigus is a real one, KS is more likely related to therapeutically induced immunosuppression in certain predisposed individuals rather than primarily to pemphigus itself.

Aged↗
PubMed1987

Plasmin induces acantholysis in skin organ cultures.

Addition of human plasminogen to three different pemphigus plasma samples showed a synergistic effect on acantholysis in the skin organ culture model. Human plasmin itself, without addition of pemphigus plasma, induced typical acantholytic changes in the skin explants, causing different types of acantholysis in a dose- and time-dependent manner: in the presence of 3 CU plasmin per ml culture medium, focal suprabasilar acantholysis of pemphigus vulgaris type could be detected after 72 h incubation, whereas 15 CU/ml caused extended acantholysis of pemphigus foliaceus type in the upper epidermal layers after 24 h, and extended acantholysis of benign chronic pemphigus (Hailey-Hailey disease) type comprising all layers of the epidermis after 48 h incubation. Plasminogen activator levels (Mr 55,000 urokinase type) in tissue extracts of skin explants and in culture media were reduced after 24 and 48 h incubation with pemphigus IgG as compared to control experiments with normal human IgG; this probably resulted from urokinase inactivation by reaction with inhibitors. These results lend support to the hypothesis proposed by Hashimoto et al. in 1983 that the plasminogen activator-plasmin system could play an essential role in the protease mechanisms of pemphigus acantholysis.

Acantholysis↗
PubMed2006

Pemphigus: a treatment update.

Pemphigus is a group of rare autoimmune mucocutaneous bullous diseases with potential significant morbidity and mortality. The two main subtypes are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Systemic corticosteroid use and other advances in management have dramatically decreased the mortality rate for pemphigus. At present, the primary cause of morbidity and mortality is complications from treatment. Thus, the goal of pemphigus management is to induce and maintain remission with the lowest possible doses of medication and with the fewest side effects. Although our scientific knowledge of pemphigus is advancing and our treatment options are expanding, there are still very few randomized, controlled studies to evaluate the true effectiveness of the available therapies. Here we review the available treatment options and novel therapies for pemphigus and the supporting data.

Animals↗
PubMed1983

Juvenile pemphigus.

Seven cases of juvenile pemphigus vulgaris and one case of juvenile pemphigus foliaceus are presented. A detailed review of the literature is also presented. The majority of patients with juvenile pemphigus develop pemphigus vulgaris. The patients present with a wide clinical spectrum. The oral cavity is frequently involved. The mean duration is 4 years. Clinical follow-up is similar to the adult variety. Several patients warrant high doses of systemic corticosteroids and develop serious side effects, most notably growth retardation and opportunistic infections. Short-term judicious use of immunosuppressive agents is advocated. Dapsone is a helpful adjuvant to therapy. It is proposed that relevant immunofluorescent studies be done very early. Early diagnosis and therapy are associated with a better prognosis.

Adolescent↗
PubMed1996

Penicillamine-induced bullous dermatoses.

The successful therapeutic use of D-penicillamine (DPA) has been hindered by its many adverse effects. Autoimmune bullous syndromes are among the less common adverse DPA reactions; they are not dose dependent and appear late in the treatment of diseases of altered immunity, most often rheumatoid arthritis. The majority of the DPA-induced bullous syndromes belong to the pemphigus spectrum, usually pemphigus foliaceus or erythematosus, have a lower prevalence of demonstrable tissue-fixed or circulating antibodies than spontaneously occurring pemphigus, display abnormal direct immunofluorescent patterns, and have a generally favorable prognosis. However, many cases do exhibit a full-blown chronic disease, unaffected by DPA withdrawal. DPA-induced cicatricial pemphigoid is a severe disease of both mucous and cutaneous involvement with a prognosis similar to the spontaneous disease. Cases of DPA-induced epidermolysis bullosa acquisita and DPA-induced bullous pemphigoid were not sufficiently substantiated by immunofluorescence or immunoprecipitation criteria.

Drug Eruptions↗
PubMed1982

Penicillamine-induced pemphigus.

Penicillamine therapy has been associated with a variety of autoimmune disorders, including pemphigus vulgaris and pemphigus foliaceus. The "late scaling rash" or oral ulcerations associated with penicillamine may represent the superficial erosions of early pemphigus. We describe four patients with pemphigus induced by penicillamine therapy and emphasize the "seborrhea-like" characteristics of this eruption that may obscure the diagnosis. A high prevalence (71%) of HLA-B15 is noted in the seven patients on whom HLA data are available. Early recognition is important because of the potentially fatal nature of pemphigus. The mechanism by which penicillamine induces or unmasks pemphigus and other immunologic syndromes remains to be elucidated.

Aged↗
PubMed1975

Pemphigus and myasthenia gravis.

Various forms of true pemphigus have been reported to occur with myasthenia gravis, with and without thymoma, more frequently than can be ascribed to chance. A57-year-old woman with myasthenia gravis in remission developed pemphigus foliaceus. No roentgenologic evidence of a thymoma was present. Antinuclear antibodies caused interference with indirect immunofluorescent determination of pemphigus antibody. Indirect immunofluorescent studies showed no evidence for cross-reactivity between intercellular substance antibody and thymic components, as is found in the case of antiskeletal muscle antibodies with thymic myoid cells. The concurrence of these two diseases may involve the failure of a subgroup of thymic-dependent lymphocytes to suppress underlying autoimmune phenomena.

Animals↗
PubMed2000

[Clinical aspects and immunopathology in 48 patients with pemphigus].

BACKGROUND AND OBJECTIVE: Pemphigus is a rare intraepidermal autoimmune bullous disease. Two major variants, pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are distinguished. The aim of this study was to document the clinical and immunopathological findings in all pemphigus patients who were diagnosed in the Department of Dermatology at the University of Würzburg over the past 10 years. PATIENTS/METHODS: Based on a retrospective study, clinical and immunopathological findings in 48 patients with pemphigus were recorded. All patients had positive findings by direct and/or indirect immunofluorescence microscopy. RESULTS: Between January 1989 and August 1998, 48 patients were diagnosed with pemphigus at our institution; 31 patients had PV and 17 PF. The average age (+/- standard deviation) of PV patients was 55 (+/- 17) and of PF patients 60 (+/- 12) years. All PV patients showed involvement of mucous membranes and in 65% of cases, the skin was also involved. In contrast, PF patients had involvement only of the skin. By direct immunofluorescence microscopy, intercellular deposits of IgG and C3 were detected in 89% and 78% of PV cases, respectively. In PF, intercellular deposits of IgG were found in 94% and of C3 in 75% of cases. By indirect immunofluorescence microscopy on monkey esophagus, 94% of the PV and 88% of the PF patients revealed circulating serum antibodies. In 30 patients, we characterized the immune response by ELISA using recombinant desmoglein 1 and 3. All PF sera showed autoantibodies against desmoglein 1 and all PV sera against desmoglein 3. In PV with both mucous membrane and skin involvement, antibodies to both desmoglein 3 and 1 were detected. CONCLUSIONS: Our results confirm the correlation of the autoantibody profile with the clinical phenotype of pemphigus.

Adult↗
PubMed1995

Conformational epitopes of pemphigus antigens (Dsg1 and Dsg3) are calcium dependent and glycosylation independent.

The target molecule of pemphigus autoantibodies is a transmembrane desmosomal component, desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and Dsg1 in pemphigus foliaceus (PF). In this study, we examined the effects of calcium and glycosylation on the anti-genicity of the pemphigus antigens and on the generation of conformational epitopes. We used recombinant baculovirus proteins, PVIg and PFIg, which are considered to reflect accurately the native conformation of the extracellular domain of their respective proteins Dsg3 and Dsg1. These baculoproteins could immunoadsorb heterogeneous autoantibodies from the corresponding sera of PV and PF patients, completely blocking indirect immunofluorescence staining of normal human skin. Chelating calcium from the solution containing the baculoproteins using ethylenediaminetetraacetic acid (EDTA) or ethyleneglycol-bis(beta-aminoethyl ether)-N,N,N',N'-tetraacetic acid (EGTA) abolished immunoadsorption by both PVIg and PFIg; however, immunoadsorption by the baculoproteins was restored after dialysis against 1 mM calcium. Nonglycosylated forms of both baculoproteins produced in the presence of tunicamycin retained their immunoadsorptive ability. Furthermore, immunoadsorption by the baculo-proteins was prevented irreversibly by treatment with low pH, high pH, and boiling, but not with the non-ionic detergent Nonidet P-40. These findings indicate that formation of the conformational epitopes on the pemphigus antigens is dependent on calcium but independent of glycosylation, and provide direct evidence that calcium plays an important role in determining the antigenic properties of the pemphigus antigens.

Adsorption↗
PubMed1991

Pemphigus in children.

Six boys with pemphigus vulgaris and one boy with pemphigus foliaceus are reported. The youngest patient had onset of the disease at the age of 7 years. Three patients with pemphigus vulgaris were treated with intravenous dexamethasone pulses, while the remaining four received oral corticosteroid therapy. The prognosis in all patients was excellent.

Adolescent↗
PubMed1993

Evidence for cell-mediated immune mechanisms in the pathology of pemphigus.

Pemphigus is an autoimmune blistering disease in which autoantibodies have clearly been shown to be pathogenic. Because autoantibodies are also found in uninvolved skin, further mechanisms may be important in the development of pemphigus lesions. In addition to granulocytes, mononuclear cells are commonly found in pemphigus lesions. To elucidate the role of mononuclear cells in the pathology of this disease, we determined the levels of soluble interleukin-2 receptor in blister fluid and serum samples from pemphigus patients prior to treatment. The interleukin-2 receptor (IL-2R) is expressed on activated mononuclear cells. Depending on its rate of synthesis, a portion is released from the cell surface in a soluble form (sIL-2R). In blister fluid, sIL-2R levels were 2186 +/- 288 U/ml (+/- SD), which was significantly higher than levels in concurrently obtained serum samples (1299 +/- 165 U/ml; P < 0.001). In suction blisters in volunteers, and in patients with second-degree burns or friction-induced bullae, sIL-2R levels were normal in both blister fluid and serum. In pemphigus patients, sIL-2R serum levels continuously declined during systemic therapy, correlating with disease activity. Immunohistological studies demonstrated a marked increase in IL-2R+ cells in both the epidermis and dermis of lesional skin compared with perilesional skin. In the dermis, CD3+ T cells predominated, whereas monocytes/macrophages were most frequent in the epidermis. In pemphigus vulgaris, monocytes/macrophages were restricted to the basal keratinocytes, whereas in pemphigus foliaceus, they were found throughout the lesional epidermis. Our data indicate that activated mononuclear cells are present in lesional skin of pemphigus patients, and may contribute to the pathology of this disease.

Aged↗
PubMed1977

An organ culture model for the study of pemphigus acantholysis.

An in vitro model for the study of pemphigus acantholysis has been developed. The histological changes of pemphigus vulgaris were reproduced in vitro in organ culture by growing normal human skin in the presence of pemphigus vulgaris or pemphigus foliaceus sera. At 24 h a suprabasilar split was noted and at 72 h extensive suprabasilar acantholysis developed. Direct immunofluorescent tests demonstrated that pemphigus antibody became bound to the epidermal intercellular space antigen(s) during the first 6-12 h. As acantholysis increased the presence of tissue-fixed antibody decreased. The fixation of the pemphigus antibody to the skin prior to the development of acantholysis provides strong evidence for the pathogenetic role of this antibody in the production of acantholysis. The data suggest that complement is not required in this model for the production of the acantholytic changes of pemphigus since heating the serum for 30 min at 56 degrees C did not destroy the acantholytic activity and no complement (C3) could be detected by DIF of organ culture explants.

Acantholysis↗
PubMed1995

A case of pemphigus vulgaris showing reactivity with pemphigus antigens (Dsg1 and Dsg3) and desmocollins.

Both pemphigus vulgaris antigen (PVA; Dsg3) and pemphigus foliaceus antigen (PFA; Dsg1) are members of the desmoglein subfamily of the cadherin supergene family. Another desmosomal cadherin, desmocollin, is occasionally recognized by certain pemphigus sera. We present a 38-year-old Japanese male who showed clinically and histopathologically typical features of pemphigus vulgaris, whose sera reacted with all PVA, PFA, and desmocollins using immunoblotting of both human epidermis and bovine snout epidermis. Studies using domain-specific fusion proteins of PFA and PVA suggested that this patient's serum reacted with the intracellular domain of PFA and the extracellular domain of PVA, the latter of which seems to be responsible for initiating the skin lesion. The patient's serum showed reactivity with human desmocollin and was shown to react with bovine Dsc2 fusion protein, further suggesting the significance of anti-desmocollin autoantibodies in pemphigus. These results indicate that certain pemphigus cases may produce antibodies against multiple antigen molecules, although the complex mechanism of the production of autoantibodies remains to be elucidated.

Adult↗
PubMed2005

A comparison of anti-desmoglein antibodies and indirect immunofluorescence in the serodiagnosis of pemphigus vulgaris.

BACKGROUND: Indirect immunofluorescence (IIF) is the standard method for the detection of pemphigus autoantibodies. Commercially available enzyme-linked immunosorbent assays (ELISAs) have recently become available to measure serum antibodies (Abs) against desmoglein1 (Dsg1) and desmoglein3 (Dsg3). It has been suggested that patients with mucosal-dominant pemphigus vulgaris (PV) have serum Abs against Dsg3 only, patients with mucocutaneous PV have Abs to both Dsg1 and Dsg3, and patients with pemphigus foliaceus (PF) have Abs against Dsg1 only. AIM: To compare the sensitivity and specificity of the IIF and ELISA tests in the diagnosis of pemphigus and its subsets. METHODS: Thirty-three patients with PV and five patients with PF were studied, and compared with 50 healthy individuals or patients with unrelated skin diseases. Monkey esophagus was used as a substrate for the IIF test. RESULTS: The IIF and ELISA tests were each positive in 26 of the 32 (81%) PV patients, and in none (0%) and 3 (6%) of the 50 controls, respectively. Both the IIF and ELISA results were concordant in 69% of the PV patients, and only one of these two tests was positive in the remaining 31% of patients. Forty-six per cent of the PV patients with a positive ELISA test did not have the PV phenotype (mucosal or mucocutaneous) predicted by their autoantibody profile. CONCLUSION: The IIF and ELISA tests may be used as complementary tests for the serologic diagnosis of pemphigus.

Adult↗
PubMed1998

Functional involvement of urokinase-type plasminogen activator receptor in pemphigus acantholysis.

Urokinase-type plasminogen activator (uPA) has been well documented in the development of pemphigus acantholysis. The function of its receptor (uPA-R) in pemphigus acantholysis has only recently attracted attention. Increased expression of uPA-R has been demonstrated in pemphigus vulgaris. In this study, we have further explored the functional involvement of uPA-R in pemphigus acantholysis. Our results show that uPA-R expression is significantly increased in acantholytic foci of pemphigus vulgaris and pemphigus foliaceus but not in bullous pemphigoid or normal skin specimens; the expression of uPA-R in cultured human keratinocytes is subjected to regulation by pemphigus vulgaris IgG but not by pemphigoid IgG or normal human IgG; furthermore, anti-uPA-R monoclonal antibody effectively inhibits pemphigus vulgaris IgG induced acantholysis in skin organ cultures. These data suggest that uPA-R may play an important role in the pathogenesis of pemphigus acantholysis.

Acantholysis↗
PubMed1998

Relationship between keratinocyte adhesion and death: anoikis in acantholytic diseases.

Loss of attachment to the substratum may trigger apoptosis in epithelial cells (anoikis). It is less clear whether apoptosis may be triggered by disruption of cell-cell contacts, as happens in acantholytic diseases. Biopsy specimens were obtained from the border of skin lesions from four patients with pemphigus vulgaris (PV), four patients with pemphigus foliaceus (PF), three patients with Darier's disease (DD), two patients with Darier's-type Grover's disease (GD), and two patients with benign familial pemphigus Hailey-Hailey disease (HH). Control skin was obtained from five healthy volunteers. TUNEL (terminal deoxynucleotidyl transferase-mediated dUTP nick end labelling) and confocal laser scanning microscopy was employed to detect the nuclei containing fragmented DNA in apoptotic cells. In PV and PF, TUNEL-stained apoptotic keratinocytes were abundantly present in the regions of acantholysis and in the cohesive epidermis below the blisters. Apoptotic keratinocytes had pyknotic, condensed nuclei. In DD, GD and HH, the number of TUNEL-stained keratinocytes was lower, apoptotic keratinocytes were confined to the regions of dyskeratosis and acantholysis, and pyknosis was absent. In conclusion, disruption of cell-cell contacts in acantholytic skin disorders may in some cases cause apoptosis of keratinocytes. Further studies are needed to determine whether the observed differences in the pattern of apoptosis are due to targeting of different junctional elements (adherens junctions in PV and PF versus desmosomes in DD, GD and HH).

Apoptosis↗