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PubMed1984

Pemphigus in the dog: comparison of immunofluorescence and immunoperoxidase method to demonstrate intercellular immunoglobulins in the epidermis.

In 3 dogs with pemphigus vulgaris and 4 dogs with pemphigus foliaceus, intercellular immunoglobulins were demonstrated in the epidermal stratum spinosum. The immunofluorescence technique on cold ethanol-fixed and paraffin-embedded tissue sections was compared with the immunoperoxidase method on formalin-fixed paraffin-embedded tissue sections. The results of both methods were identical. However, the advantage of the unlabeled antibody-enzyme method was that the same formalin-fixed tissue specimens could be used for conventional light microscopy, as well as for immunohistologic studies.

Animals↗
PubMed2003

Pemphigus erythematosus associated with anti-DNA antibodies and multiple anti-ENA antibodies: a case report.

Pemphigus erythematosus (PE) is an autoimmune blistering disease combining features of pemphigus foliaceus (PF) and systemic lupus erythematosus (SLE). We report a case of PE associated with anti-double-stranded DNA (anti-dsDNA), anti-Smith (anti-Sm), anti-Ro (anti-SSA), and antiribonucleoprotein (anti-RNP) antibodies. This case required extensive immunosuppressive therapy. We treated our patient with a combination of systemic steroids, intramuscular gold injections, azathioprine, and hydroxychloroquine. The patient's response was complete remission--evaluated clinically, serologically, and immunohistochemically.

Adult↗
PubMed1984

[Brazilian pemphigus in a child in French Guyana. Discussion on the clinical polymorphism and epidemiology of the disease].

The observation in French Guyana of pemphigus foliaceus having long mimicked eczema or psoriasis and turning 5 year later as an exudative erythroderma, points out the clinical polymorphism of the disease. Several clinical features are analysed that might support the hypothesis of autonomy of the Brazilian type: such as for example joint involvement. But the main point concerns epidemiology since this disease seems to spread out from the classical endemic Brazilian provinces to northern countries as socio-economical conditions improve in the south.

Administration, Topical↗
PubMed1979

Comparison of different epithelial substrates useful for indirect immunofluorescence testing of sera from patients with active pemphigus.

A positive statistical correlation between intercellular antibody titer and disease severity was found for a group of 6 patients with active pemphigus vulgaris, irrespective of whether indirect IF testing was performed on 5 different epithelial substrates which included guinea pig esophagus, human foreskin, rabbit esophagus, monkey esophagus and monkey lip. In a group of 4 patients with pemphigus foliaceus a positive correlation between disease severity and titer was found only when rabbit esophagus was used for indirect fluorescence testing. In both groups of patients there were individual patients in whom the antibody titer was positive at a time when no disease was present and conversely, there were some patients in whom the antibody titer was negative at a time when extensive disease was present. Because of this inconsistency, the use of antibody titers to monitor disease activity and therapy in individual patients may not be justifiable.

Animals↗
PubMed2006

Prevalence of anti-desmoglein-3 antibodies in endemic regions of Fogo selvagem in Brazil.

Fogo selvagem (FS), the endemic form of pemphigus foliaceus (PF), is an autoimmune blistering disease characterized by autoantibodies against desmoglein 1. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a previously identified focus of disease. Autoantibodies against desmoglein 3 (Dsg3) have also been detected in sera from patients with FS. In an effort to further characterize the serological, geographical, and clinical epidemiology of the disease, we sought to determine the prevalence of anti-Dsg3 autoantibodies in sera from normal subjects living outside of and in an endemic area using an ELISA. Anti-Dsg3 antibodies were detected in 53 of 146 normal subjects from Limao Verde (36%), and in eight of 140 normal subjects from surrounding areas (6%). A significant trend was observed in the proportion of positive tests relative to distance from the endemic area (P < 0.001). Our seroepidemiological observations support the concept that the likely environmental trigger of the antibody response in FS is located in this endemic area, and that the population at risk to develop FS may also be at risk to develop an endemic form of pemphigus vulgaris as reported by our co-investigators from Brasilia.

Autoantibodies↗
PubMed1993

Concomitant occurrence of circulating IgA anti-intercellular and anti-basement membrane zone antibodies in autoimmune blistering diseases: immunofluorescence and immunoblot studies.

Recently, cases with circulating IgA anti-intercellular antibodies have been described. The objective of this study was to present immunofluorescence and immunoblot findings in three cases of bullous diseases with concomitant circulating IgA anti-intercellular and anti-basement membrane zone antibodies. Direct immunofluorescence, indirect immunofluorescence on intact and 1M NaCl-split skin, immunoblotting of epidermal extracts from dispase- and EDTA-separated (two different procedures) human skin, and immunoblotting of the bovine desmosome preparation were performed. All three cases had IgA anti-intercellular and anti-basement membrane zone antibodies. However, immunoblot results were divergent. Case 1 had antibodies against the 150 kD pemphigus foliaceus antigen (IgG), the 170 kD protein (IgG and IgA), and the 97 kD antigen (IgG and IgA). Case 2 had IgG antibodies reactive with the 230 kD and the 170 kD bullous pemphigoid antigens, while case 3 had IgA antibodies against the 97 kD antigen only. The results of immunofluorescence and immunoblot studies in our patients widen the spectrum of laboratory features in blistering skin diseases mediated, at least in part, by antibodies of the IgA class.

Adult↗
PubMed1998

Erythroderma: a clinico-etiologic study of 90 cases.

BACKGROUND: The difficulty with erythroderma lies in finding the underlying cause. Almost all the published original clinical series of erythroderma originate from western countries. Our aim was to evaluate various causes of exfoliative dermatitis in our community and compare the findings with previous studies. METHODS: Ninety patients with erythroderma of either sex and any age, were studied at the Dermatology Department, Mayo Hospital, Lahore, Pakistan, between April 1992 and May 1995. A through clinical examination and relevant laboratory investigations, including skin biopsies, were performed. RESULTS: The mean age of onset was 41.6 years with a male:female ratio of 2.8:1. The onset was acute in 69% of patients. The salient features included nail changes (80%), mucosal involvement (36.6%), alopecia (30%), islands of normal skin (14.4%), and the "deck chair sign" (5.5%). The most frequent cause of erythroderma was pre-existing dermatoses (74.4%), including psoriasis (37.8%), dermatitis (12.2%), ichthyoses (7.8%), and pemphigus foliaceus (5.6%). Drugs and malignancy each induced erythroderma in 5.5% of cases. No cause could be found in 14.6% of cases. CONCLUSIONS: Etiologically, pre-existing dermatoses showed the highest incidence and drugs the lowest compared with previous studies. Dermatitis was less common. There was a greater variety of causes of erythroderma in our series. Hair and nails were more frequently involved. Mucosal involvement, not mentioned in other studies, was present in 36.6% of our cases. The "deck chair sign" and islands of normal skin were seen in dermatoses not reported previously.

Adolescent↗
PubMed1993

Dynamic expression of pemphigus and desmosomal antigens by cultured keratinocytes.

Dynamic expression of pemphigus antigens by cultured human and mouse keratinocytes was compared with that of desmosome-associated molecules and cellular markers relating to epidermal differentiation. Plakoglobin was detected in localized areas of keratinocyte sheets in low Ca2+ (0.15 mM) KGM medium. In minimum essential medium (MEM) containing 1.8 mM Ca2+, plakoglobin was expressed in the intercellular spaces (ICS) throughout the keratinocyte sheet. Desmoplakin I and II, which were present in the cytoplasm of keratinocytes in the low Ca2+ medium, moved to the cell surface after the medium was changed to MEM. Desmoglein 1 and pemphigus vulgaris (PV) antigens were observed in the ICS of both the monolayers and stratified areas in the MEM. Pemphigus foliaceus (PF) antigens, frequently together with desmoglein 1, involucrin and keratins specific for the upper layer of the epidermis, were expressed by stratified keratinocytes but not the cells in the monolayers. The Western blotting study of the cultured keratinocyte extract showed 160- and 130-kDa bands positive for desmoglein 1 antigens and a 130-kDa band stained with PV sera. These findings suggest that although desmoglein 1 molecules bear PF antigenic sites, their expression pattern by cultured keratinocytes is closely related to that of PV rather than PF antigens. The PF antigenic sites may be formed on desmoglein 1 during epidermal differentiation.

Animals↗
PubMed1994

Regulation of pemphigus and desmosomal antigen expression by keratinocyte differentiation.

We studied in vivo binding sites of pemphigus antibodies and the expression of pemphigus and desmosomal antigens by keratinocytes in various culture periods. Both pemphigus vulgaris (PV) and pemphigus foliaceus (PF) antibodies mainly bound to the desmosomal areas of the lesional skin. Desmoglein and PV antigens co-localized on the cultured normal human keratinocytes in both monolayers and stratified areas. PF antigens, frequently together with involucrin and suprabasal keratins, were expressed by stratified keratinocytes. The Western blotting study demonstrated two different desmogleins with molecular sizes of 130 and 150 kD. The 130-kD desmoglein bore PV antigenic epitopes. The 150-kD band increased in volume as cultured keratinocytes stratified. PV antigen expression on the 130-kD desmoglein precedes PF antigen formation occurring in keratinocyte differentiation.

Antigen-Antibody Reactions↗
PubMed2004

Diffuse cutaneous candidiasis in a dog. Diagnosis by PCR-REA.

The authors describe a clinical case of cutaneous candidiasis in a dog with dermatological lesions, characterized by persistent alopecia, crusts, ulcers and scales. Predisposing factors such as the use of corticosteroids, the concomitan presence of an autoimmune disease (pemphigus foliaceus) and an infection of ehrlichiosis caused by Ehrlichia canis were observed. Histopathological findings included signs of orthokeratotic hyperkeratosis, moderate follicular keratosis and light epidermic acanthosis. The reactive process included an infiltrative superficial dermatitis and a mural folliculitis with prevalent participation of macrophages and lymphocytes. The application of PCR-Restriction Enzyme Analysis (REA) method on cutaneous specimens in veterinary medicine is an extremely interesting diagnostic tool. Its use, together with other techniques, such as mycologic, cytologic and histological examinations, allowed us to identify Candida albicans as aetiological agent in this particular case.

Adrenal Cortex Hormones↗
PubMed1991

Distinct types of IgG and IgA anti-intercellular autoantibodies from patients with pemphigus and vesiculopustular dermatosis.

The pattern of binding of two different types of IgA class pemphigus-like antibodies was compared with that of the true IgG class pemphigus antibody. The IgG antibodies from pemphigus vulgaris (PV) and pemphigus foliaceus (PF) sera bound to the intercellular spaces in normal human epidermis, whereas only the PV antibody reacted with monolayers of keratinocytes derived from human foreskin. Both IgG and IgA antibodies from a patient with PF were found in the intercellular spaces of the epidermis and only the IgA antibody reacted with the cultured keratinocytes. The IgA antibody from a patient with a vesiculopustular eruption and whose serum contained IgA intercellular space antibody alone, bound to the upper epidermis but not to the monolayers of keratinocytes. These findings indicate that PV but not PF antigens can be expressed by monolayers of cultured human keratinocytes and that there are at least two distinct types of IgA intercellular antibodies.

Autoantibodies↗
PubMed2005

Anti-CD20 monoclonal antibody (rituximab) in the treatment of pemphigus.

BACKGROUND: Pemphigus is a severe autoimmune blistering disorder caused by autoantibodies to desmoglein 1 and 3. The disease course is typically severe, thus requiring multiple immunosuppressive agents. The treatment is still challenging and in some patients with recalcitrant disease, therapies fail and therapeutic options are limited. OBJECTIVES: To investigate whether depletion of B lymphocytes that are thought to produce disease-causing autoantibodies shows a long-term benefit in pemphigus. METHODS: Five patients diagnosed as having pemphigus vulgaris and pemphigus foliaceus were treated with the monoclonal antibody rituximab. Rituximab was administered intravenously at a dosage of 375 mg m(-2) once weekly for 4 weeks. RESULTS: The treatment was well tolerated and all patients showed a good response over a follow-up period of up to 3 years, allowing immunosuppressive treatment to be reduced or terminated. B-cell depletion persisted for 6-12 months, and in one patient for almost 3 years. CONCLUSIONS: This study highlights the prolonged effect and disease control after one single course of rituximab and further extends the spectrum of treatments of bullous autoimmune disorders.

Adult↗
PubMed2004

Immunohistochemical examination of P-cadherin in bullous and acantholytic skin diseases.

Autoimmune blistering diseases (pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, dermatitis herpetiformis) and certain genodermatoses with acantholysis (Darier-disease, Hailey-Hailey disease) have different aetiological factors, but all result in bulla formation and/or in acantholysis. Cadherins are Ca++-dependent cell-cell adhesion molecules which play an important role in the cellular connection between normal cells. P-cadherin is involved in the selective adhesion of epidermal cells, and is expressed only on the surfaces of the two basal layers. We examined the expression of P-cadherin in some autoimmune bullous skin diseases and Darier's disease using immunohistochemistry and found P-cadherin to be strongly upregulated. We believe the upregulation is compensatory to the primary pathophysiological events in the various bullous dermatoses.

Acantholysis↗
PubMed1983

Normal human skin echogram.

Sector B-mode echograms of the stratum corneum from the top of pemphigus foliaceus bulla, 15/1,000 inch split-thickness skin graft including the epidermis and superficial dermis, and the whole skin with subcutaneous fat were compared with histologic sections. The corneo-epidermal and epidermo-dermal interfaces were not delineated either by 5, 10 or 15 MHz transducer. The subcutaneous fat exhibited an almost echolucent zone with weak echoes from the fibrous trabeculae or large blood vessels. Regional differences of in vivo normal skin echograms were studied on the forehead, abdomen, back, upper arm, thigh, lower leg and heel. The presence or absence of the cutaneous muscles, fascia or bone was clearly demonstrated. For the standard echogram of the skin, a 10 MHz transducer was considered appropriate, though the detailed echograms were obtained mostly in the deep dermal and subcutaneous structures.

Adipose Tissue↗
PubMed2000

Bovine gingival lysate: a novel substrate for rapid diagnosis of autoimmune vesiculo-bullous diseases. A preliminary observation.

Immunoblot assays have been developed to characterize the autoantigens and to detect autoantibodies in muco-cutaneous autoimmune vesiculo-bullous diseases using different substrates. However the results have been inconsistent, because availability and standardization of different substrates has been a major problem. The aim of this study was to develop an immunoblot assay using bovine gingival lysate as substrate because it is easily and readily available as well as inexpensive. Sera from patients with different vesiculo-bullous diseases were studied. These included 25 patients with pemphigus vulgaris (PV), 8 with paraneoplastic pemphigus (PNP), 12 with pemphigus foliaceus (PF), 25 with bullous pemphigoid (BP), and 22 with cicatricial pemphigoid (CP). Serum samples from 40 normal human volunteers were also studied. The autoantibody titers were determined based on the binding pattern of each disease and compared to those obtained by routine indirect immunofluorescence (IIF). Our observations suggest that the titers from immunoblot assays were significantly higher than titers obtained by IIF (P<0.0001). When the autoantibody titers were compared using bovine gingival lysate and human epidermal lysate as substrate, statistically significant differences were not observed. The use of bovine gingival lysate as a substrate will facilitate the rapid and early serological diagnosis of patients with vesiculobullous diseases. It may also be of benefit to laboratory investigators studying these autoantibodies.

Animals↗
PubMed1998

Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that occurs in association with underlying neoplasms. Patients with PNP develop characteristic IgG autoantibodies directed against multiple antigens, most of which have been identified as cytoplasmic proteins of the plakin family (desmoplakin I, II, BPAG1, envoplakin, and periplakin). This study identified cell surface target antigens of PNP. We focused on desmoglein (Dsg) 3 and Dsg1, the autoantigens of pemphigus vulgaris and pemphigus foliaceus. ELISA using baculovirus-expressed recombinant Dsgs (rDsg3, rDsg1) has revealed that 25 out of 25 PNP sera tested were positive against Dsg3 and 16 of 25 were positive against Dsg1. All of 12 PNP sera tested immunoprecipitated Dsg3. Removal of anti-Dsg3 autoantibodies by immunoadsorption was sufficient to eliminate the ability of PNP sera to induce cutaneous blisters in neonatal mice in vivo. Furthermore, anti-Dsg3-specific antibodies that were affinity purified from PNP sera were proven to be pathogenic and caused blisters in neonatal mice. These findings indicate that Dsg3 and Dsg1 are the cell surface target antigens in PNP and that IgG autoantibodies against Dsg3 in PNP sera play a pathogenic role in inducing loss of cell adhesion of keratinocytes and causing blister formation.

Acantholysis↗
PubMed1997

Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins.

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune skin diseases caused by autoantibodies against desmoglein (Dsg) 3 and Dsg1, respectively. Routine immunofluorescence testing of skin and serum from patients cannot distinguish between these two severe diseases since both have IgG Abs directed against keratinocyte cell surfaces. In this study, recombinant Dsg3 and Dsg1, produced as secreted proteins by baculovirus expression, have been utilized to develop ELISAs for the specific characterization of their autoantibodies. Of 49 PV sera, 46 were positive in the Dsg3 ELISA and 44 of 46 PF sera were positive in the Dsg1 ELISA, compared with only 3 of 23 sera of bullous pemphigoid, and none of 53 normal control sera in both ELISAs. Both the Dsg3 and Dsg1 ELISAs were more specific and sensitive than conventional immunofluorescence staining. These Ag-specific ELISAs revealed that more than one-half of PV sera (26 of 49) had anti-Dsg1 Abs in addition to anti-Dsg3 Abs. PV patients who had not only oral mucous lesions but also significant skin involvement tended to have higher titers of anti-Dsg1 Abs. Furthermore, the ELISA reactivity correlated well with clinical disease activity in 5 of 6 PV and 5 of 5 PF patients. This ELISA provides a sensitive and highly specific assay for the diagnosis of patients with PV and PF, the correlation of disease activity with serum Ab levels, and a novel tool for investigating the immunopathogenesis of pemphigus.

Autoantibodies↗
PubMed2006

Synergistic pathogenic effects of combined mouse monoclonal anti-desmoglein 3 IgG antibodies on pemphigus vulgaris blister formation.

Pemphigus vulgaris (PV) is an autoimmune blistering disease caused by anti-desmoglein 3 (Dsg3) IgG antibodies. Previously, we generated an active mouse model for PV by adoptive transfer of splenocytes from immunized or naive Dsg3(-/-) mice. In this study, we isolated 10 anti-Dsg3 IgG mAbs (NAK-series) from PV model mice generated by transfer of naive Dsg3(-/-) splenocytes. We characterized their epitopes using domain-swapped and point-mutated Dsg1/Dsg3 molecules and examined their pathogenic activities in blister formation in three different assays. In a passive transfer model using neonatal mice, eight of 10 NAK mAbs showed pathogenic activity when injected together with half the minimum pathogenic dose of anti-Dsg1 IgG autoantibodies from pemphigus foliaceus (PF) patients. None of the mAbs could induce the PV phenotype when individual hybridoma clones were inoculated by peritoneal injection into adult Rag2(-/-) mice. NAK mAbs displayed a range of potency in an in vitro dissociation assay using primary cultured mouse keratinocytes. Interestingly, when multiple hybridoma clones recognizing different epitopes were inoculated in combination, recipient mice developed the PV phenotype. In vitro dissociation assays confirmed that combined NAK mAbs had synergistic pathogenic effects. These findings indicate that although an individual anti-Dsg3 IgG is not sufficient to cause blistering in adult mice, several together can induce the PV phenotype. These mAbs will provide a valuable tool to investigate the molecular mechanisms of blister formation, mimicking the effects of the polyclonal IgG antibodies found in patients.

Aging↗