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PubMed2001

Increased antibody levels to desmogleins 1 and 3 after administration of carbamazepine.

Desmoglein (Dsg) 1 and Dsg3 are recognized as the autoantigens in pemphigus foliaceus and pemphigus vulgaris. Pemphigus-like syndromes have been reported to occur in individuals after exposure to a variety of drugs, but pemphigus caused by carbamazepine is not common. We found that anti-Dsg1 and anti-Dsg3 antibody titres were increased in three individuals administered carbamazepine. Antibody titres against Dsgs 1 and 3 were measured by enzyme-linked immunosorbent assay (ELISA). Of 42 serum samples (25 patients administered carbamazepine, eight patients administered valproic acid and nine healthy volunteers) tested by ELISA, three patients administered carbamazepine showed positive reactivity against both Dsg1 and Dsg3. The patient with the highest titre against Dsg1 and Dsg3 (the index values of anti-Dsg1 and anti-Dsg3 were 79.3 and 86.4, respectively) was a 23-year-old woman (Case 1). The other two patients with positive reactivity were a 5-year-old boy (Case 2) and a 65-year-old man (Case 3). In addition, indirect immunofluorescence study showed intercellular antibodies to the cell surface of the whole epidermis with a titre of 1 : 64 in Case 1 and 1 : 2 in Cases 2 and 3. However, no skin or mucosal involvement was found in any of these cases. There was no difference in the serum concentrations of carbamazepine between the three positive cases and the 22 negative cases of carbamazepine administration. From these facts, the lack of skin diseases may be explained by relatively low values of anti-Dsg 1 and 3 antibodies in Cases 2 and 3. However, it cannot be excluded that undefined exogenous and/or endogenous factors are involved in an outbreak of pemphigus. Furthermore, these findings might be helpful for preventing susceptible individuals from exposure to the suspect drugs.

Adolescent↗
PubMed2004

Mechanisms of blister formation by staphylococcal toxins.

Many children suffer from the bacterial skin diseases bullous impetigo and staphylococcal scalded skin syndrome (SSSS). Staphylococcus aureus, which produces exfoliative toxins (ETs), causes these diseases. Recently, it was proven that ETs cleave the cell adhesion molecule desmoglein (Dsg) 1, which plays an important role in maintaining the proper structure and barrier function of the epidermis. Surprisingly, Dsg1 is also the antibody target in the autoimmune disease pemphigus foliaceus. Skin biopsies from pemphigus foliaceus patients show the same pathology as those from bullous impetigo and SSSS patients. The crystal structure of ET suggests that it is a serine protease with an inactive catalytic site, which may become activated when ET binds a specific receptor. This receptor binding is thought to cause a change in conformation that exposes the catalytic site. It has recently been shown that Dsg1 specifically binds and activates ET, which in turn cleaves the bound Dsg1 at only one peptide bond. This process is absolutely dependent on the calcium-dependent conformation of Dsg1. These data suggest that ETs have a very high specificity for human Dsg1, and that S. aureus uses ETs to disrupt the barrier of the human epidermis in order to survive and proliferate on the human body.

Blister↗
PubMed1997

cDNA cloning of the 210-kDa paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex.

Although the 210 and 190-kDa proteins are the most frequently detected antigens reacting with sera of patients with paraneoplastic pemphigus (PNP) in immunoblot analysis, there is still uncertainty as to the nature of these PNP antigens. To isolate and characterize a cDNA clone encoding the 210-kDa PNP antigen, we screened a human keratinocyte lambda gt 11 cDNA expression library by the immunoperoxidase method with serum IgG from a PNP patient. The IgG used for the immunoscreening of a keratinocyte cDNA expression library recognized 210- and 190-kDa antigens by immunoblotting. A single clone, called here the PNP clone, producing a fusion protein that reacted strongly with the patient's IgG, was further characterized. Only the PNP patient's IgG, but not IgG from a normal control, pemphigus foliaceus, or pemphigus vulgaris patients, bound the plaques of this positive clone. Furthermore, PNP IgG affinity purified on plaques of this clone, but not unrelated clones, bound to keratinocyte cell surfaces by immunofluorescence and reacted with the 210-kDa PNP antigen by immunoblotting. EcoRI digestion of the clone's cDNA insert demonstrated a 1.4-kbp fragment. This cDNA insert was placed into a M13 mp 18 vector and sequenced. Sequence analysis revealed that the cDNA insert of the PNP clone encodes a part of the central rod domain and the COOH-terminal C domain of envoplakin, a newly defined precursor of the cornified envelope that is homologous to desmoplakin. This result demonstrates that the 210-kDa PNP antigen is envoplakin and PNP is an autoimmune disease that produces autoantibodies against intermediate filament-associated proteins in desmosomes and hemidesmosomes, desmoplakin, bullous pemphigoid antigen 1 (BPAG 1), and envoplakin.

Autoantibodies↗
PubMed1993

Immunofluorescence and immunoelectron microscopy analyses of a human monoclonal anti-epithelial cell surface antibody that recognizes a 185-kD polypeptide: a component of the paraneoplastic pemphigus antigen complex?

We recently reported the production of a human monoclonal antibody (MoAb) derived from a patient with pemphigus vulgaris (PV) that binds to the keratinocyte membrane and reacts with a 185-kD polypeptide by immunoblot analysis. We have since examined the tissue specificity of that MoAb, F12. By indirect immunofluorescence (IIF), F12 stained both the cell membrane and the basement membrane zone of stratified squamous epithelia. Moreover, MoAb F12 stained other epithelial tissues, such as urinary bladder, small bowel, thymus, and liver, and non-epithelial tissues, such as myocardium. Indirect immunoelectron microscopy (IIEM) analysis showed that MoAb F12 bound to a component common to desmosomal and hemidesmosomal plaques and to zona adherens-type junctions between hepatocytes and bile duct cells. Inhibition experiments were then performed with sera from patients with pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, or bullous pemphigoid. Three sera blocked F12 reactivity; two were from paraneoplastic pemphigus patients and the other was from the pemphigus vulgaris patient whose peripheral blood lymphocytes were used to make F12. All these sera recognized a 185-kD band that co-migrated with the polypeptide labeled by MoAb F12 on immunoblots. In addition, the IIF and IIEM staining patterns of MoAb F12 were similar to those observed with sera from two patients with paraneoplastic pemphigus. These observations suggest a relationship between MoAb F12 and the autoimmune response characterizing paraneoplastic pemphigus patients' sera.

Antibodies, Monoclonal↗
PubMed2006

Antidesmoplakin antibodies in pemphigus vulgaris.

BACKGROUND: Besides being present in paraneoplastic pemphigus (PNP), circulating antidesmoplakin (DP) antibodies have been found anecdotally in other bullous diseases, including pemphigus foliaceus and pemphigus vulgaris. OBJECTIVES: To verify how frequent anti-DP antibodies are in pemphigus vulgaris. METHODS: We studied 48 sera from patients with proven pemphigus vulgaris (29 mucosal dominant pemphigus and 19 mucocutaneous pemphigus) by indirect immunofluorescence (IIF) with rat bladder epithelium (RBE) as a substrate and by immunoblotting (IB) on human keratinocyte cultures enriched in DP. RESULTS: Ten sera (21%) were positive in IIF on RBE. By IB, eight sera proved to have antibodies to both DP I (250 kDa) and DP II (210 kDa), one serum had antibodies directed to DP I only, and two sera to DP II only. CONCLUSIONS: Our data confirm that RBE is not a specific IIF substrate for the serological diagnosis of PNP. It remains a sensitive and specific substrate for the detection of anti-DP antibodies, which, in patients with pemphigus vulgaris, are probably caused by an epitope-spreading phenomenon.

Animals↗
PubMed2004

Pemphigus: current therapy.

Pemphigus is an autoimmune skin disease that can present in a variety of forms and can be a challenging disease to manage and treat. An overview of the different forms of pemphigus and diagnostics are discussed including pemphigus foliaceus (PF), pemphigus erythematosus (PE), panepidermal pustular pemphigus (PPP), pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP). Emphasis on therapy is presented. Included are the most current commonly used therapeutics (glucocorticoids, azathioprine, chlorambucil and tetracycline and niacinamide); current alternative therapeutics (cyclosporin and tacrolimus and mycophenolate mofetil) and additional alternative therapeutics (cyclophosphamide, chrysotherapy, dapsone, sulfasalazine and intravenous immunoglobulin (IVIG) therapy).

Animals↗
PubMed1997

Expression of desmogleins in Paget cells of mammary and extramammary Paget's disease.

Sagebiel (1969) electronmicroscopically observed that desmosomes are present between adjacent Paget cells, as well as between Paget cells and adjacent keratinizing epidermal cells. Desmosomes contain the proteins desmoglein (Dsg) and desmocollin as their transmembrane components, and Dsg has three isotypes. Among the three isotypes of Dsg, Dsg1 and Dsg3 are autoantigens for pemphigus foliaceus and pemphigus vulgaris (PV), respectively. We examined the expression of Dsgs in Paget cells of mammary and extramammary Paget's disease. Skin samples were obtained from one patient with mammary Paget's disease, and from 2 with extramammary Paget's disease. One part of the samples was cut into small pieces and epidermal sheets were separated with dispase, then treated with a mixture of EDTA and trypsin. The resulting cell suspensions were cultured in low Ca++ medium, then the cells were incubated in high Ca++ medium. Paget cells identified with anti-epithelial membrane antigen (EMA) antibody were positive for serum from a PV patient which was confirmed to react with both Dsg1 and Dsg3. However, the intensity of fluorescence of Paget cells was weaker than that of EMA-negative keratinocytes. In the cryostat sections, Paget cells identified with anti-EMA antibody showed the same staining pattern as cultured cells in high Ca++ medium. The data presented in this study confirm that Paget cells express Dsgs, and are consistent with the electronmicroscopical data by Sagebiel (1969). However, our data do not support the hypothesis that Paget cells are of keratinocyte origin, because sweat ducts or sweat glands could be positive for sera from PV patients. It is necessary to confirm whether or not sweat ducts or sweat glands express Dsgs, because sera from PV patients exhibit high background in the dermis.

Aged↗
PubMedDate not supplied

[The significance of desmosomes in pathology].

Considerable progress has been made in our knowledge of desmosomes and their components. Molecular cloning of the desmosomal glycoproteins has established that desmoglein 1 and desmoglein 3 are targets for autoantibodies in the blistering diseases pemphigus foliaceus and pemphigus vulgaris respectively. New evidence suggests that another desmosomal glycoprotein, desmocollin 1, is the major target antigen in the upper epidermal form of intercellular IgA dermatosis. In human cancer there is accumulating evidence which suggests a role for desmosomes in the prevention of the invasion and metastasis. The possibility exists that a mutation in a desmosomal glycoprotein gene is responsible for an inheritable human disease, the striated form of palmoplantar keratoderma.

Animals↗
PubMed2004

Pemphigus.

Pemphigus vulgaris and the related pemphigus vegetans, pemphigus foliaceus and pemphigus erythematous, are associated with the highest mortality among primary skin diseases. Typical findings are illustrated.

Disease Progression↗
PubMed1996

[Cell adhesion molecules and extracellular matrix components as target structures of autoimmunity].

A number of cell-cell and cell-matrix adhesion molecules as well as several extracellular matrix components represent target structures of antibody-mediated autoimmunity which recently have been extensively characterized at the molecular biological level. Pathogenic autoantibodies against these molecules have been found to be causally related to disturbances of cell and tissue adhesion that become apparent as various (muco-)cutaneous blistering diseases. In desmosomes, desmosomal cadherins (desmogleins and desmocollins) mediate epidermal intercellular adhesion. Among these, desmoglein 1 and desmoglein 3 are the autoantigens of pemphigus foliaceus and pemphigus vulgaris, respectively, being characterized by intraepidermal blistering. The pathogenic relevance of autoantibodies against desmocollins (IgA pemphigus and other pemphigus types) and desmoplakins (paraneoplastic pemphigus) still remains unclear. Hemidesmosomes contain the plaque protein BPAG1 and the partly collagen-like transmembrane protein BPAG2, representing the autoantigens of bullous pemphigoid and pemphigoid gestations which show subepidermal blistering. A certain subtype of cicatricial (benign mucous membrane) pemphigoid is characterized by autoantibodies against laminin 5 present in the subhemidesmosomal anchoring filaments. Both epidermolysis bullosa acquisita and bullous SLE exhibit autoantibodies against collagen type VII which constitutes the anchoring fibrils. Besides, autoantibodies against a particular collagen type IV chain of the glomerular basement membrane are responsible for the manifestation of Goodpasture's syndrome. These recent molecular biological findings might be the basis for the development of novel therapeutic strategies.

Animals↗
PubMed1997

Desmosomes and disease.

Considerable progress has been made in our knowledge of desmosomes and their components. Molecular cloning of the desmosomal glycoproteins has established that desmoglein 1 and desmoglein 3 are targets for autoantibodies in the blistering diseases pemphigus foliaceus and pemphigus vulgaris respectively. New evidence suggests that another desmosomal glycoprotein, desmocollin 1, is the major target antigen in the upper epidermal form of intercellular IgA dermatosis (IgA pemphigus). In human cancer there is accumulating evidence which suggests a role for desmosomes in the prevention of invasion and metastasis. The possibility exists that a mutation in a desmosomal glycoprotein gene is responsible for an inheritable human disease, the striated form of palmoplantar keratoderma.

Animals↗
PubMed1990

Immunoperoxidase staining for the detection of autoantibodies in canine autoimmune skin disease; comparison to immunofluorescence results.

Skin sections from 22 dogs with autoimmune skin disease were stained with anti-canine IgG, IgM and IgA using an immunobridge immunoperoxidase method. Eight cases of lupus erythematosus, three cases of pemphigus vulgaris, and 11 cases of pemphigus foliaceus were included. Results of previously performed, direct immunofluorescence tests for the detection of canine immunoglobulin on skin were available on 17/22 cases. The immunoperoxidase method yielded an overall positive result in 59% (5/8 lupus erythematosus, 2/3 pemphigus vulgaris and 6/11 pemphigus foliaceus) versus an overall positive result of 47% for direct immunofluorescence (3/5 lupus erythematosus, 2/2 pemphigus vulgaris and 2/10 pemphigus foliaceus). The immunobridge immunoperoxidase method compared favorably to direct immunofluorescence testing of canine skin for autoantibody in cases of lupus erythematosis and pemphigus vulgaris, and was superior in cases of pemphigus foliaceus. This method should prove useful as an aid in the diagnosis of canine autoimmune skin disease.

Animals↗
PubMedDate not supplied

[Treatment of severe refractory pemphigus vulgaris with rituximab].

Pemphigus vulgaris is a potentially fatal autoimmune bullous disease. High doses of immunosuppressive drugs are used in managing severe cases of pemphigus. Rituximab, an anti-CD20 monoclonal antibody, has proven to be effective in patients with refractory pemphigus vulgaris and pemphigus foliaceus. We review cases of pemphigus vulgaris and pemphigus foliaceus not associated with lymphoma that were treated with rituximab, and we report a new case of severe refractory pemphigus vulgaris successfully treated with rituximab.

Antibodies, Monoclonal↗
PubMed2006

A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus.

OBJECTIVE: To investigate the safety and efficacy of oral methylprednisolone combined with azathioprine sodium or mycophenolate mofetil for the treatment of pemphigus. DESIGN: A prospective, multicenter, randomized, nonblinded clinical trial to compare 2 parallel groups of patients with pemphigus (pemphigus vulgaris and pemphigus foliaceus) treated with oral methylprednisolone plus azathioprine or oral methylprednisolone plus mycophenolate mofetil. Settings Thirteen departments of dermatology in Germany. Patients We included patients with pemphigus vulgaris (n = 33) or pemphigus foliaceus (n = 7) evidenced by clinical lesions suggestive of pemphigus, intraepidermal blistering on histological analysis of skin biopsy specimens, intercellular deposition of IgG within the epidermis, and immunoblot analysis findings for antidesmoglein 3 and/or antidesmoglein 1 autoantibodies. MAIN OUTCOME MEASURES: The cumulative total methylprednisolone doses and rate of remission. Secondary outcome measures were safety profiles and duration of remission. RESULTS: In 13 (72%) of 18 patients with pemphigus receiving oral methylprednisolone and azathioprine, complete remission was achieved after a mean +/- SD of 74 +/- 127 days compared with 20 (95%) of 21 patients receiving oral methylprednisolone and mycophenolate mofetil in whom complete remission occurred after a mean +/- SD of 91 +/- 113 days. The total median cumulative methylprednisolone dose used was 8916 mg (SD, +/-29 844 mg) in the azathioprine group compared with 9334 mg (SD, +/-13 280 mg) in the mycophenolate group. In 6 (33%) of 18 patients treated with azathioprine, grade 3 or 4 adverse effects were documented in contrast to 4 (19%) of 21 patients who received mycophenolate mofetil. Conclusion Mycophenolate mofetil and azathioprine demonstrate similar efficacy, corticosteroid-sparing effects, and safety profiles as adjuvants during treatment of pemphigus vulgaris and pemphigus foliaceus.

Administration, Oral↗
PubMed2001

Dual diagnosis of Pemphigus and pemphigoid. Retrospective review of thirty cases in the literature.

BACKGROUND: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. OBJECTIVE: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. METHODS: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. RESULTS: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. CONCLUSION: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.

Adult↗
PubMed1999

Childhood pemphigus.

BACKGROUND: Five children with pemphigus are reported: three with pemphigus vulgaris, one with pemphigus vegetans, and one with pemphigus foliaceus. Only one case of juvenile pemphigus vegetans has been published in the literature. MATERIALS AND METHODS: All three patients with pemphigus vulgaris were treated with oral corticosteroid; in two cases, azathioprine was added for steroid-sparing effect. The patient with pemphigus vegetans had a clinical presentation resembling pemphigus vulgaris, but the lesions in the perianal area healed as hypertrophic granulation tissue. He was treated with oral corticosteroid, azathioprine, and intralesional corticosteroid. The patient with pemphigus foliaceus presented with exfoliative dermatitis, and was treated with oral corticosteroid; methotrexate was added later for steroid-sparing purposes RESULTS: The patients were followed up for 1-4 years; the prognosis of childhood pemphigus is good. CONCLUSIONS: Long-term follow-up is needed to detect flaring of the disease and the side-effects of immunosuppressive drugs.

Adolescent↗
PubMed1996

Neutrophilic spongiosis in pemphigus.

BACKGROUND: Acantholysis is the histologic hallmark in the diagnosis of all forms of pemphigus. However, biopsy specimens of early lesions may lack acantholysis and show only eosinophils in the epidermis in areas of spongiosis (eosinophilic spongiosis). We report two cases of pemphigus foliaceus and two cases of unclassified pemphigus (foliaceus vs vulgaris) in which neutrophilic spongiosis was the prominent histologic finding. OBSERVATIONS: Four patients developed blistering skin disorders that spared the mucous membranes. Skin biopsy specimens in all four patients showed striking infiltration of neutrophils into the epidermis. Acantholysis was focal and was absent in some sections. Direct immunofluorescence demonstrated intercellular deposition of IgG and C3 within the epidermis in all cases. There was no IgA deposition. Gram's stains were negative for bacteria in three cases and revealed Gram-positive cocci overlying an eroded area in one case. However, the neutrophilic spongiosis in this case extended well beyond the area of impetiginization. CONCLUSIONS: The histologic differential diagnosis of neutrophils in the epidermis includes pustular psoriasis, subcorneal pustular dermatosis, intraepidermal neutrophilic IgA dermatosis, superficial IgA pemphigus, toxic shock syndrome, Sweet's syndrome, and superficial fungal and bacterial infections. We conclude that pemphigus be added to this differential diagnosis and recommend direct immunofluorescence when neutrophilic spongiosis is observed.

Acantholysis↗
PubMedDate not supplied

Rare clinical form in two patients affected by a new variant of endemic pemphigus in northern Colombia.

BACKGROUND: Recently, the authors described a new variant of endemic pemphigus foliaceus in rural areas surrounding El Bagre, Colombia, but without association with malignant tumors. METHODS: The authors' 10-year fieldwork provided the opportunity to observe various manifestations of El Bagre endemic pemphigus foliaceus, including the presence of bilateral plaques in pretibial areas. RESULTS: Based on personal experience and literature reviews, the authors have correlated the auto-antibody profile with the appearance of pretibial plaques. CONCLUSION: Since pretibial plaques occur in patients with both fogo selvagem and El Bagre variants of endemic pemphigus foliaceus, as well as in other forms of pemphigoid, these diseases must be considered in the differential diagnosis of patients with clinical, immunologic, and/or epidemiologic risk factors.

Adult↗