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PubMed1994

[Pemphigus].

Pemphigus is a rare autoimmune bullous disease of the skin and mucosae. It has predisposing genetic factors and, in the case of pemphigus foliaceus, environmental factors; some therapeutic drugs may trigger off pemphigus. The disease often begins with painful buccal erosions, followed by flaccid bullae on healthy skin. Some lesions are erythematous plaques involving the seborrheic areas. Cytology shows acantholysis and histology, intraepidermal cleavage. Direct immunofluorescence confirms the diagnosis by showing a network deposit of immunoglobulins and complement. The titres of circulating antibodies to the intracellular substance vary with the course of the disease. The loss of cohesion between keratinocytes is induced by autoantibodies directed against antigens of the epidermal cell junction zone; the molecular weight of these antibodies is 130 and 85 kD for pemphigus vulgaris and 160 and 85 for superficial pemphigus. Paraneoplastic pemphigus is a recently individualized entity. Treatment consists of systematic corticosteroid therapy, sometimes associated with immunosuppressants; it has improved the prognosis of this once lethal disease.

Humans↗
PubMed1987

Effects of pemphigus antibody on the regeneration of cell-cell contact in keratinocyte cultures grown in low to normal Ca++ concentration.

Pemphigus is an autoimmune blistering disease of epidermal cells in which autoantibodies to the surface develop. The present study was performed to determine whether the binding of pemphigus antibodies to the surface of keratinocytes can inhibit the regeneration of cell-cell contact induced by altering from low to normal Ca++ concentration medium. Human keratinocytes (a cell line of squamous cell carcinoma, DJM-1 cell) were grown in low Ca++ medium for 4 days, then the cells were incubated in normal Ca++ medium containing 10% pemphigus (4 patients with pemphigus vulgaris and 4 patients with pemphigus foliaceus) or normal serum (treated at 56 degrees C, for 30 min) for various incubation periods (2, 6, 12, 24 h). The cells were fixed and stained with antikeratin antibody by the indirect immunofluorescence method so that the detachment of cell-cell contact was able to be clearly visualized by observing the cytoskeletal arrays of keratin filaments. The cells grown in normal Ca++ medium showed detachments of cell-cell contact 24-36 h after addition of any one of the pemphigus sera used in this study. The cells grown in low Ca++ medium formed no cell-cell contacts and expressed no pemphigus antigens. However, re-formation of cell-cell contacts and reexpression of the antigens were confirmed by immunofluorescence microscopy 30 min after the addition of Ca++ to the medium. The addition of any pemphigus vulgaris and foliaceus sera with Ca++ did not inhibit the regeneration of cell-cell contact and exerted no effects on the contact during the subsequent 12 h. However, after 24 h, these cells again lost the contact. These results indicate that pemphigus antibody and antigen reaction on the cell surface did not directly inhibit the Ca++-induced re-formation of cell-cell contact.

Antibodies↗
PubMed1999

[Autoimmune bullous skin diseases].

INTRODUCTION: Important advances have been made in the pathogenesis of autoimmune bullous skin diseases during the last 10 years. The aim of this review is to describe new types of autoimmune bullous skin diseases and to summarize progresses in the pathogenesis and treatment of these diseases. CURRENT KNOWLEDGE AND KEY POINTS: Paraneoplastic pemphigus (PNP) is a new type of pemphigus, distinct from pemphigus vulgaris (PV) and pemphigus foliaceus (PF). It has both clinical and histological features of pemphigus, bullous pemphigoid (BP), erythema multiforme, and lichen planus, in patients with lymphoma. Numerous clinical types of autoimmune bullous skin diseases of the dermal epidermal junction have also been described. Analysis of patients' serum by immunoblotting and immunoprecipitation allowed the identification of target antigens of most of these diseases: PV and PF are due to the production of autoantibodies directed against adhesion molecules (desmoglein 1 and 3) that belong to the cadherin family. In PNP, autoantibodies are directed against plakins, a new family of desmosomal plaque proteins. BP, CP and pemphigoid gestationis are characterized by the production of autoantibodies mainly directed against two hemidesmosomal proteins: BPAG1 and BPAG2. The mortality rate of autoimmune bullous skin diseases is estimated between 10 and 40%, mainly due to infections and cardiovascular diseases. The potential interest of the first use of adjuvant therapies in addition to corticosteroids has not been demonstrated yet. FUTURE PROSPECTS AND PROJECTS: Due to the numerous side-effects of oral corticosteroids, many therapeutic trials are aimed at finding other treatments for the management of these diseases. Topical corticosteroids could be effective in BP, and even in some cases of pemphigus. New treatments using specific immunoadsorption of pathogenic autoantibodies with recombinant proteins will probably be available in the future.

Autoantibodies↗
PubMed2001

Paraneoplastic pemphigus sera react strongly with multiple epitopes on the various regions of envoplakin and periplakin, except for the c-terminal homologous domain of periplakin.

Paraneoplastic pemphigus sera react with multiple plakin family proteins, among which only envoplakin and periplakin are constantly detected by immunoblotting using normal human epidermal extracts. Using bacterial expression vectors containing polymerase chain reaction-amplified cDNA, we have prepared variously truncated recombinant glutathione-S-transferase-fusion proteins of envoplakin and periplakin, which presented N-terminal, central and C-terminal domains of each protein, as well as the so-called C-terminal homologous domain of envoplakin and the junctional regions of these domains. By immunoblotting using these 11 recombinant proteins, we demonstrated that most of the 26 paraneoplastic pemphigus sera reacted very strongly with multiple recombinant proteins of envoplakin and periplakin, except for the C-terminal homologous domain of periplakin. We also examined the reactivity with these recombinant proteins of other blistering diseases, including pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, and found that a few nonparaneoplastic pemphigus sera showed a weak reactivity with some of the recombinant proteins. Interestingly, some sera showed relatively strong reactivity with the C-terminal homologous domain of periplakin to which paraneoplastic pemphigus sera reacted less frequently. These results indicate that, although nonparaneoplastic pemphigus sera occasionally show a weak reactivity with envoplakin and periplakin, the pathogenicity and the mechanism of antibody production in these cases may be different from those in paraneoplastic pemphigus.

Autoimmune Diseases↗
PubMed2004

New approaches to the treatment of pemphigus.

In pemphigus vulgaris, treatment with systemic glucocorticosteroids is life saving; it may, however, cause severe side effects, including death. A patient with pemphigus vulgaris and myasthenia gravis was treated for approximately five years with the cholinomimetic Mestinon (pyridostigmine bromide), Imuran (azathioprine), and a topical corticosteroid gel before the need to introduce systemic glucocorticosteroids. Because activation of keratinocyte acetylcholine receptors also has been shown to abolish pemphigus IgG-induced acantholysis in cultured keratinocyte monolayers, a clinical trial of Mestinon was initiated in patients with active pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic autoimmune multiorgan syndrome (also known as paraneoplastic pemphigus). First results indicate that nonsteroidal treatment of pemphigus is possible. Mestinon may be used to slow down progression of the disease and to treat mild cases with chronic lesions on limited areas. Stimulation of the keratinocyte- acetylcholine axis may lead to a therapeutic effect through any of the following mechanisms: (1) stimulating keratinocyte cell-to-cell attachment; (2) accelerating reepithelialization; and (3) competing with the disease-causing pemphigus antibodies, preventing them from attachment to keratinocytes. Glucocorticosteroids and various types of steroid-sparing drugs used to treat pemphigus exhibit cholinergic side effects, including effects on expression and function of keratinocyte adhesion molecules, that are very similar to those produced by the cholinomimetic drugs. Further elucidation of the mechanisms underlying therapeutic efficacy of antiacantholytics may shed light on the immunopharmacological mechanisms of pemphigus antibody-induced acantholysis.

Administration, Topical↗
PubMed1983

Diagnosis of autoimmune skin disease in the dog: correlation between histopathologic, direct immunofluorescent and clinical findings.

Histopathologic, immunopathologic and clinical evaluation of two hundred thirty dogs resulted in a diagnosis of canine autoimmune skin disease (AISD) in eighty four of the suspected cases. Pemphigus foliaceus was the most common diagnosis, followed by bullous pemphigoid and pemphigus vulgaris. The highest percentage of cases were mixed breeds, followed by shelties, collies, German shepherds and poodles. There was no apparent age or sex predilection. Eighty seven percent of the eighty four cases of AISD were diagnosed on the basis of supportive clinical criteria in addition to compatible histopathologic and/or direct immunofluorescent findings. Thirteen percent of the cases with a final clinical diagnosis of AISD lacked supportive immunohistopathologic criteria on biopsy and the diagnosis was based upon clinical appearance, exclusion of other dermatoses, and response to immunosuppressive therapy. This latter group included four cases of SLE diagnosed using a combination of clinical criteria and a positive test for antinuclear antibody. Thirty three percent of the AISD cases were diagnosed on the basis of compatible findings on both histopathology and direct immunofluorescence. A histopathologic diagnosis of AISD was made in sixty nine percent of the cases. Positive direct immunofluorescence was found in fifty two percent of the cases. Thirty five percent of the AISD cases were diagnosed on histopathologic criteria without supportive immunofluorescent findings, while nineteen percent had positive direct immunofluorescence with nonspecific histopathologic changes. The number of definitive positive results was greater for direct immunofluorescence (38%) than for histopathology (25%). There was an equal incidence (21%) of false positive results among non-AISD cases with both tests. Indirect immunofluorescence appears to have little value in the diagnosis of canine AISD. The recommended diagnostic approach to canine AISD involves the use of both histopathologic and immunopathologic evaluations.

Animals↗
PubMed2002

Pemphigus vulgaris localized to the nose and cheeks.

Pemphigus vulgaris is an autoimmune blistering disease characterized by disseminated bullae and erosions of the mucosal surfaces and skin. Cases of pemphigus vulgaris with localized lesions on glabrous skin have been reported, and most of them have been attributed to the Koebner phenomenon. Lesions limited to the nose have mostly been described in pemphigus foliaceus, but the nose has also been the initial site of pemphigus vulgaris in a few patients. We report 4 cases of localized pemphigus vulgaris: one case with lesions limited to the nose and cheeks and 3 cases with isolated lesions on the nose. Recurrent episodes in these patients also occurred on the nose. None of them showed mucosal involvement or dissemination during a follow-up period of 2 to 9 years. Three patients had autoantibodies against desmoglein-3 as detected by immunoblot. In the other patient, antibodies against desmoglein-3 were detected by enzyme-linked immunosorbent assay. These localized lesions may represent a subgroup of pemphigus vulgaris or a period of limited activity during this chronic disease.

Adult↗
PubMed2000

Sensitivity and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemphigus.

BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease characterized by the production of autoantibodies mainly directed against proteins of the plakin family. An overlapping distribution of autoantibody specificities has been recently reported between PNP, pemphigus vulgaris (PV), and pemphigus foliaceus (PF), which suggests a relationship between the different types of pemphigus. OBJECTIVE: Our purpose was to evaluate the sensitivity and the specificity of clinical, histologic, and immunologic features in the diagnosis of PNP. METHODS: The clinical, histologic, and immunologic features of 22 PNP patients were retrospectively reviewed and compared with those of 81 PV and PF patients without neoplasia and of 8 PV and 4 PF patients with various neoplasms. RESULTS: One clinical and 2 biologic features had both high sensitivity (82%-86%) and high specificity (83%-100%) whatever the control group considered: (1) association with a lymphoproliferative disorder, (2) indirect immunofluorescence (IIF) labeling of rat bladder, and (3) recognition of the envoplakin and/or periplakin bands in immunoblotting. Two clinicopathologic and two biologic features had high specificity (87%-100%) but poor sensitivity (27%-59%): (1) clinical presentation associating erosive oral lesions with erythema multiforme-like, bullous pemphigoid-like, or lichen planus-like cutaneous lesions; (2) histologic picture of suprabasal acantholysis with keratinocyte necrosis, interface changes, or lichenoid infiltrate; (3) presence of both anti-epithelial cell surface and anti-basement membrane zone antibodies by IIF; and (4) recognition of the desmoplakin I and/or BPAG1 bands in immunoblotting. Interestingly, 45% of patients with PNP presented initially with isolated oral erosions that were undistinguishable from those seen in PV patients, and 27% had histologic findings of only suprabasal acantholysis, which was in accordance with the frequent detection of anti-desmoglein 3 antibodies in PNP sera. CONCLUSION: The association with a lymphoproliferative disorder, the IIF labeling of rat bladder, and the immunoblotting recognition of envoplakin and/or periplakin are both sensitive and specific features in the diagnosis of PNP.

Humans↗
PubMed1980

An immunofluorescence study of light chain in pemphigus.

Immunofluorescence studies were made on light (L) chain of immunoglobulins in patients with pemphigus. These patients fell into two groups: a subcorneal bulla group, consisting of 10 patients with Senear-Usher syndrome, and one with pemphigus foliaceus, and a suprabasal bulla group consisting of 8 patients with pemphigus vulgaris. In the former group there was deposition of L chains of either type alone in the intercellular spaces of the upper epidermis: kappa type alone in 9 and lambda type alone in 2 patients. Deposition of L chains of both types was observed in the intercellular spaces of the lower epidermis in all patients of the latter group. No significant difference existed between groups as to the titre of circulating intercellular antibodies of either type. Some comments are made on the significance of the L chain of immunoglobulins in a pemphigus.

Adolescent↗
PubMed1994

Detection of anti-desmocollins I and II autoantibodies in two cases of Hallopeau type pemphigus vegetans by immunoblot analysis.

Pemphigus foliaceus (PF) sera react with a 150 kDa antigen, desmoglein (DG), while pemphigus vulgaris (PV) sera react with a 130 kDa PV antigen. Recent molecular cloning studies have revealed that both DG and PV antigen are members of the cadherin family of cell adhesion molecules and that PV antigen shows very high homology to DG. We have recently noticed that other desmosoal cadherin molecules, desmocollins I and II (DC I/II), are also recognized by some pemphigus antibodies. In the present study we report two cases of Hallopeau type pemphigus vegetans. Immunoblot study revealed that the sera of these cases reacted not only with the PV antigen but also with both DG (PF antigen) and DC I/II. Although the significance of these findings is not known at present, further studies for the antigen profile for more Hallopeau type pemphigus vegetans patients may unravel the pathogenesis of this rare type of pemphigus.

Adult↗
PubMed1987

Calcium enhances the sensitivity of immunofluorescence for pemphigus antibodies.

Indirect immunofluorescence (IF) to detect pemphigus and pemphigoid autoantibodies is commonly performed with monkey esophagus (ME) as substrate and phosphate-buffered saline (PBS) as a diluent. The purpose of this study was to evaluate comparative IF titers using human skin (HS) as substrate with variations in the buffers employed. Substrates (ME or HS) were incubated in PBS, Tris-acetate-buffered saline (TAS), TAS with 5 mM CaCl+2 (TAS-Ca+2), and PBS or TAS with 1 mM EDTA, prior to incubation with pemphigus or pemphigoid sera for indirect IF. We examined sera from 11 patients with pemphigus vulgaris (PV), 10 patients with Brazilian pemphigus foliaceus (BPF), and 4 patients with bullous pemphigoid. In 20 of 21 pemphigus sera, endpoint indirect IF titers were highest on normal skin with TAS-Ca+2. Six sera (2 PV and 4 BPF) had endpoints that were 5 double dilutions higher than the endpoints obtained with ME and PBS. Six sera (3 PV and 3 BPF) were 4 double dilutions higher, 7 sera (3 PV and 4 BPF) were 2-3 double dilutions higher, and 2 PV sera were equivalent with both substrate/buffers. Preincubation of either tissue with EDTA prior to indirect IF abolished PV and BPF antibody binding completely. Exposure to EDTA after the tissue was incubated with PV or BPF sera did not affect indirect IF titers. In the presence of Ca+2, the antigen was resistant to trypsin in concentrations of 0.001%; however, in the absence of added Ca+2 it was destroyed by 0.0001% trypsin. These differences were not observed with bullous pemphigoid sera; all 4 sera had similar endpoint indirect IF titers. This study shows a significant increase in the sensitivity of indirect IF assays for pemphigus autoantibodies by the use of Ca+2-supplemented buffers on human skin. This finding may also have implications for procedures designed to purify and/or detect pemphigus antigens.

Adult↗
PubMed1991

Autoantigens for IgA anti-intercellular antibodies of intercellular IgA vesiculopustular dermatosis.

A new disease characterized by the presence of in vivo bound and/or circulating IgA anti-intercellular (IC) antibodies has recently been identified. We propose the term intercellular IgA vesiculopustular dermatosis (IAVPD) for this entity, which seems to be divided clinicopathologically into at least two distinct subtypes: intraepidermal neutrophilic IgA dermatosis (IEN type) and subcorneal pustular dermatosis-like cases (SPD type). Using immunoblot technique, we examined the antigen substances for the IgA anti-IC antibodies in the sera from one Japanese patient with IEN type of IAVPD and three Japanese patients with SPD type. A serum from a patient with IEN type reacted exclusively with a 120-kD protein in both the normal human skin extract and the bovine desmosome sample. Sera from three patients with SPD type reacted specifically with a doublet of 115-kD and 105-kD proteins, which appeared to be identical to desmocollins I and II, well known desmosomal core proteins, in the bovine desmosome sample. IgA antibody from our patients with IAVPD bound to neither pemphigus vulgaris antigen nor pemphigus foliaceus antigen. From these results, we suggest that IAVPD is different from pemphigus and is heterogeneous in terms of the antigens to which IgA autoantibodies bind.

Autoantibodies↗
PubMed1993

Paraneoplastic pemphigus: a subset of patients with pemphigus and neoplasia.

We reviewed the clinical, histologic, and immunofluorescence features of 20 patients with pemphigus and neoplasia and compared them with a control group of 17 patients with pemphigus without neoplasia. Patients with neoplasia were divided according to clinical, histologic, and immunofluorescence findings into those with paraneoplastic pemphigus syndrome (12 patients) and those with classic pemphigus vulgaris or pemphigus foliaceus with neoplasia (8 patients). The histologic findings in patients with paraneoplastic pemphigus included acantholysis, interface dermatitis, spongiosis, and satellite keratinocyte necrosis. Histologic findings in the 8 patients with classic pemphigus and neoplasia included acantholysis and spongiosis. Direct immunofluorescence in both paraneoplastic pemphigus and pemphigus with neoplasia showed IgG staining of cell-surface proteins (intercellular substance) and deposition of immunoglobulin at the basement membrane zone. Indirect immunofluorescence with rat bladder substrate was used to differentiate paraneoplastic pemphigus from classic pemphigus. Circulating IgG anti-cell-surface protein antibodies were detected in 4 patients with paraneoplastic pemphigus syndrome; they were absent in 2 patients with pemphigus and neoplasia. Immunoprecipitation of sera from the 4 patients with epithelial staining showed the complex of bands identified in studies of paraneoplastic pemphigus syndrome. We conclude that paraneoplastic pemphigus syndrome has distinct clinical, histologic, and immunologic features that differentiate it from classic pemphigus with underlying neoplasia.

Aged↗
PubMed1996

Specific detection of anti-cell surface antibodies in herpes gestationis sera.

We examined 42 herpes gestationis sera with immunofluorescence of normal human skin sections, and found that anti-keratinocyte cell surface antibodies were detected specifically in 10 herpes gestationis sera. The diagnosis of these herpes gestationis cases was confirmed by detecting antibodies against the 180 kD bullous pemphigoid antigen with immunoblotting of its fusion protein. The results of immunoadsorption assay using baculoproteins of both pemphigus vulgaris and pemphigus foliaceus antigens indicated that the herpes gestationis sera did not recognize common pemphigus antigens. Immunoblotting of human epidermal extracts and immunofluorescence of various tissues also suggested that the sera did not recognize any other desmosomal components or paraneoplastic pemphigus antigens. The significance of this reactivity is unclear. However, because no control bullous pemphigoid sera showed this reactivity, it may suggest a different pathophysiology between herpes gestationis and bullous pemphigoid.

Antigens, Surface↗
PubMed1991

Calcium metabolism and the pathogenesis of dermatologic disease.

Calcium has a major role in regulating epidermal functions, including cell proliferation, terminal differentiation, and cell-to-cell adhesion. Aberrations in calcium regulation have been noted in psoriasis when levels of the calcium binding protein calmodulin are elevated, and the normal calcium gradient within the epidermis is altered. Calcium may also be important in neoplasia because similar elevations in calmodulin have been noted in transformed cells. The function of calcium in mediating cell-cell adhesion may be important in understanding the acantholysis observed in pemphigus. The pemphigus foliaceus antigen appears to contain a calcium-sensitive epitope, and in pemphigus vulgaris, alteration in the function of calcium-sensitive cadherins may play a role in the production of acantholysis. Further understanding of the function of calcium in these processes may, in the future, allow us to alter calcium metabolism as a therapeutic intervention.

Animals↗
PubMed1995

Adhesion molecules. I: Keratinocyte-keratinocyte interactions; cadherins and pemphigus.

During the last few years, considerable progress has been made in our understanding of the structure and function of cadherins and of the pathophysiology of pemphigus. Cadherins are a multiple gene family of Ca(++)-dependent cell adhesion molecules with a typical single-spanning transmembrane structure. Cadherins have two major subfamilies, classic cadherin and desmosomal cadherin. Classic cadherins, including E-, P-, and N-cadherins, are characterized by a homophilic binding specificity. They localize at adherens junctions and mediate physiologic interaction with the involvement of cytoplasmic anchoring molecules, catenins, and the actin-based cytoskeleton network. Desmosomal cadherins, the desmocollins and desmogleins, localize at desmosomes and are linked to the intermediate keratin filaments network via plakoglobin and desmoplakin. Molecular cloning has demonstrated that the autoantigens of both pemphigus vulgaris and pemphigus foliaceus are members of the desmoglein subfamily of the cadherin supergene family. Thus, pemphigus is characterized as an anti-cadherin autoimmune disease. Furthermore, a baculovirus recombinant protein of pemphigus vulgaris antigen was capable of absorbing out the pathogenic autoantibodies from patients' sera, providing a possibility of antigen-specific therapeutic strategies for pemphigus.

Cadherins↗
PubMed1994

Pemphigus developed on preexisting dermatoses.

A case of pemphigus foliaceus which initially developed at sites of preexisting psoriasis and a case of pemphigus erythematosus that developed on the lesions of photosensitive erythemas are described. Inflammatory dermatoses can be considered to be local factors which facilitate the autoimmune response of pemphigus, which in turn may induce blistering cutaneous lesions in subclinical patients with autoantibodies.

Adult↗
PubMed1995

Differences in the expression of pemphigus antigens during epidermal differentiation.

Epidermal desmogleins with molecular weights of 130/140 kDa (Dsg3 or PVA) and 150/160 kDa (Dsg1 or DGI) are recognized by autoantibodies from patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF), respectively. In order to understand the histogenesis of both types of pemphigus, we studied the expression patterns of Dsg1 and Dsg3 during stratification of cultured keratinocytes. Monolayers of cultured normal human keratinocytes demonstrated uniform intercellular staining with PV sera. The staining pattern was distinct from the focal staining with PF sera observed only in the stratified areas. Both Dsg1 and Dsg3 proteins and their mRNA were expressed by the monolayers, whereas no production of Dsg2 (HDGC) mRNA was found. The relative ratio of Dsg3 to the total desmogleins, as determined by density on immunoblotting, decreased as the cultured keratinocytes stratified. In the completely stratified keratinocytes cultured on collagen membrane, Dsg1 became predominant, with subsequent reduction of PV antigen expression. The relative decrease of Dsg3 (PVA) during epidermal differentiation might be responsible for the induction of suprabasal acantholysis in PV.

Autoantigens↗