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PubMed2003

Oral cyclophosphamide for treatment of pemphigus vulgaris and foliaceus.

BACKGROUND: Cyclophosphamide is an alkylating adjuvant used in refractory cases of pemphigus. OBJECTIVE: We sought to evaluate the effectiveness and safety of oral cyclophosphamide in the treatment of patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) with refractory disease. PATIENTS: We studied 23 patients with pemphigus (20 with PV; 3 with PF) who failed to achieve clinical remissions with the use of prednisone and antimetabolites. RESULTS: Complete remission was achieved in 17 patients with PV and 2 with PF. A total of 3 patients with PV failed therapy. A partial remission was achieved in 1 patient with PF. The treatment was administered for a median duration of 17 months with a follow-up period of 27 months. The median time to complete remission was 8.5 months. A total of 9 patients who were severely affected received concomitant plasma exchange. Adverse reactions included 5 cases of hematuria, 6 nonlife-threatening infections, and the development of transitional cell carcinoma of the bladder 15 years after discontinuation of cyclophosphamide in 1 patient. No death was associated with cyclophosphamide treatment. CONCLUSION: Oral cyclophosphamide is an effective adjuvant in the treatment of severe and refractory PV and PF, but requires close monitoring.

Administration, Oral↗
PubMed1996

Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies.

We have examined autoantigens for IgA anti-keratinocyte cell surface antibodies in 17 intercellular IgA vesiculopustular dermatosis (IAVPD) cases showing only IgA antibodies and 5 cases showing both IgG and IgA antibodies (G A cases). IAVPD cases were divided into two subtypes: (1) intraepidermal neutrophilic IgA dermatosis type showing pustule formation throughout the epidermis and IgA antibodies reactive with the entire epidermis and (2) subcorneal pustular dermatosis type containing IgA antibodies reactive with the uppermost portion of the epidermis. Most G A cases showed atypical clinical features. With immunoblot analysis of normal human epidermal extracts, IgA antibodies in these cases showed no specific reactivity with either pemphigus foliaceus antigen (desmoglein 1) or pemphigus vulgaris antigen (desmoglein 3), except that IgC antibodies in one G/A case each recognized one of the two antigens. With immunoblotting of desmosome enriched fraction obtained from bovine snout epidermis, IgA antibodies in 10 IAVPD and 3 G/A cases and IgG antibodies in 4 G/A cases showed reactivity with either desmoglein 1 or desmocollin another desmosomal cadherin. These results indicate that IAVPD and G/A cases are heterogeneous in terms of both clinical features and antigens and that the IgA autoantibodies in these cases may react with different antigens from those for IgG autoantibodies.

Animals↗
PubMed1999

Immunoblot and immunoelectronmicroscopic analysis of endemic Tunisian pemphigus.

Tunisian pemphigus is a newly described form of endemic pemphigus whose clinical, histological and epidemiological characteristics have recently been detailed. The objective of this study was to analyse the binding properties of autoantibodies present in sera from patients with endemic Tunisian pemphigus using immunoblotting and indirect immunoelectron microscopy (IEM). Thirty patients with pemphigus foliaceus (PF) and six with pemphigus vulgaris (PV) seen in the dermatology department of Tunis Hospital between 1992 and 1994 were selected for this study. Seven of 30 (23%) and six of 12 (50%) PF sera tested bound to the 160 kDa band of desmoglein 1 when tested on bovine tongue and human epidermal extracts, respectively. Two of six and two of three PV sera tested bound to the 130 kDa desmoglein 3 in these two extracts. Immunoblot and indirect IEM showed that 24 of 30 (80%) PF sera contained IgG1, IgG3 or IgG4 antibodies that bound to a 185-kDa polypeptide localized on the desmosomal plaque. This immunological analysis showed that most endemic Tunisian pemphigus sera correspond to PF sera and are characterized by a high frequency of autoantibodies directed against a recently identified 185-kDa antigen of the desmosomal plaque.

Adult↗
PubMed1978

Serum immunoglobulin E in pemphigus.

Immunoglobulin E levels in the sera of patients with pemphigus (12 with Brazilian pemphigus foliaceus (BPF) and 11 with pemphigus vulgaris (PV)) were determined by means of a solid phase radioimmunoassay. A significant increase in IgE level was observed in BPF patients compared to the level of IgE in PV patients and healthy adults. The implications of an elevated level of IgE with respect to other aberrations of immunologic responsiveness and the suggested infectious etiology of BPF are considered.

Complement C3↗
PubMed1994

Pemphigus: past, present and future.

Pemphigus remains the archetype of the autoimmune bullous diseases with circulating and skin antibodies in patients as well as in animal models. Pemphigus antigens are desmosomal-associated glycoproteins of 130 kD in pemphigus vulgaris (desmoglein 3) and of 160 kD (desmoglein 1) in pemphigus foliaceus (fogo selvagem in Brazil, pemphigus seborrheicus in other countries). Pemphigus may be associated to drugs and/or immune system related tumors (e.g. thymoma). Two new forms of pemphigus have been described: IgA pemphigus and paraneoplastic pemphigus. Targets of the autoantibodies are various desmosomal and cellular adhesion molecules. Even if each main pemphigus variant corresponds to specific antibodies, association of various antibodies may be found, leading to the concept of dysimmunoreactivity.

Animals↗
PubMed2004

Defining the pathogenic involvement of desmoglein 4 in pemphigus and staphylococcal scalded skin syndrome.

Desmogleins (Dsgs), cadherin-type cell adhesion molecules, are targeted in skin-blistering diseases such as pemphigus and staphylococcal scalded skin syndrome (SSSS). The role of Dsg4, a new isoform, was investigated in these diseases. Dsg4 was recognized by 30 (77%) of 39 pemphigus sera containing anti-Dsg1 IgG but not by 16 pemphigus sera containing no anti-Dsg1 IgG or by 34 normal control sera. The Dsg4 immunoreactivity of these sera was abolished by removal of anti-Dsg1 IgG. Conversely, the removal of anti-Dsg4 IgG from pemphigus sera reduced the immunoreactivity against Dsg1 only 13.8% +/- 8.8% (n = 23) and did not affect its ability to induce blisters in neonatal mice. IgG that was affinity-purified on Dsg4 recognized Dsg1 but failed to induce blisters, while IgG purified on Dsg1 from the same pemphigus foliaceus sera induced blisters. Thus, pemphigus sera show Dsg4 reactivity due to cross-reactivity of a subset of anti-Dsg1 IgG, and the Dsg4/Dsg1-cross-reacting IgG has no demonstrable pathogenic effect. In addition, Dsg4 was not cleaved by exfoliative toxins that induce blisters in SSSS. These findings suggest that Dsg4 may play a role other than adhesion and that the cross-reactivity of desmoglein autoantibodies should be factored into the framework of future studies of autoimmune mechanisms in pemphigus.

Amino Acid Sequence↗
PubMed1996

Bilateral herpes simplex virus keratitis in a patient with pemphigus vulgaris.

Pemphigus is a group of chronic blistering diseases in which acantholysis and blister formation occur within the epidermis. Immunoglobulins and complement are found in the circulation and are bound to the cell surfaces of keratinocytes. Pemphigus is classified into several types but may be divided into two major variants, pemphigus vulgaris and pemphigus foliaceus. The primary skin lesion of pemphigus vulgaris which was often fatal before the introduction of systemic glucocorticoid therapy, is a flaccid, fragile blister which can occur anywhere. The most common skin lesions are erosions, which are often painful; suprabasal clefting within the epidermis is also present. In the majority of these patients, painful mucous membrane erosions will be the first symptom, while sometimes the conjunctiva is affected but corneal involvement is very rare. There are few reports of herpes simplex keratitis occurring with a blistering disease. This is a case report of bilateral herpes simplex virus keratitis superinfection during the glucocorticoid treatment of pemphigus vulgaris.

Aged↗
PubMed1991

[Polymorphisms of HLA class II antigens in 33 Japanese pemphigus patients].

The HLA class II antigens in 33 Japanese pemphigus patients were investigated by both serologic and restriction fragment length polymorphism (RFLP) analyses: 17 cases of pemphigus vulgaris (PV), 13 cases of pemphigus foliaceus (PF), 3 cases of unclassified pemphigus. In serologic typing, DR2 was absent in PV. DR5, DRw6, DRw12, and DRw52 were positively associated with PV. DQw1 was positively associated with PF. RFLP analyses showed that DRw6 PV patients had a disease-associated restriction fragment representing DQw5, the same association as that found in DRw6 Jewish PV patients. On the other hand, all 13 PF patients were serologically typed for DQw1, which could not be further subdivided into DQw5 by RFLP analyses. These results suggest that Japanese and Jewish PV patients may be immunogenetically closely related to each other, but Japanese PV patients appear to be immunogenetically different from Japanese PF patients.

Asian People↗
PubMed1996

Pemphigus in Mali: a study of 30 cases.

Pemphigus has been largely studied in developed countries (North America and Europe) and in Brazil. In these geographical settings, pemphigus presents two very different epidemiological and clinical patterns. Little is known about pemphigus in other regions of the world, particularly in Africa. We report here a study of 30 cases of pemphigus observed in Bamako, Mali. Our data suggest that pemphigus in this area presents a distinctive pattern. Our cases of pemphigus were diagnosed on the basis of clinical, histological and direct immunofluorescence studies. We estimated the annual incidence in the Bamako region to be 0.29 cases per 100,000 inhabitants. There was no endemic focus in Mali. The disease was observed mainly in women (24 of 30; 80%), especially those older than 40 years (mean age, 46.7 years), and in the Fulani ethnic group (10 of 30; 33%). Our study group was composed of 25 cases of pemphigus foliaceus (PF) (83%), four cases of pemphigus vulgaris and one case of pemphigus vegetans. Pustules with hypopyon were observed in 11 patients (37%). A diffuse verrucous change in the skin was noted in four cases of erythrodermic PF. In 16 patients with PF, localized verrucous lesions mimicking seborrhoeic keratoses were observed when oral corticosteroid treatment was decreased. Histopathological examination demonstrated eosinophilic spongiosis in 50% of patients. These data suggest that pemphigus in Mali differs from the two main known patterns of the disease: the North American/European one, and the Brazilian pattern, with which it shares the predominance of superficial forms but otherwise differs in many features.

Adult↗
PubMed2004

Kaposi's varicelliform eruption in association with rosacea.

Kaposi's varicelliform eruption is characterized by disseminated vesiculopustules and erosions caused by a herpes virus infection superimposed on a pre-existing dermatosis. The eruption usually occurs in individuals with atopic dermatitis or other pre-existing dermatosis such as Darier's disease, pemphigus foliaceus, mycosis fungoides, Sezary syndrome, benign familial pemphigus, ichthyosis vulgaris, second-degree burns, multiple myeloma, and Grover's disease. We report here a new case of Kaposi's varicelliform eruption in a 38-year-old woman with rosacea. To our knowledge, this is the first case of Kaposi's varicelliform eruption associated with rosacea to be reported.

Adult↗
PubMed1999

Intercellular IgA dermatosis (IgA pemphigus)--two cases illustrating the clinical heterogeneity of this disorder.

IgA pemphigus is rare but may be underdiagnosed. We describe two cases, a 50-year-old female with a pustular eruption resembling subcorneal pustular dermatosis and a 55-year-old male with a pruritic vesiculopustular eruption simulating dermatitis herpetiformis. They illustrate the clinical heterogeneity of IgA pemphigus which is likely to reflect differences in autoantigens, analogous to pemphigus vulgaris and pemphigus foliaceus. There is now evidence that IgA pemphigus encompasses at least two subgroups: a subcorneal pustular dermatosis (SPD)-type, (see case 1) characterized by subcorneal pustules and autoantibodies to desmocollin 1; and intra-epidermal neutrophilic dermatosis (IEN)-type cases (see case 2) which show intra-epidermal pustules and in whom the autoantigen may be desmoglein 3, the pemphigus vulgaris antigen.

Anti-Inflammatory Agents↗
PubMed1995

Mucocutaneous paraneoplastic manifestations of hematologic malignancies.

PURPOSE: To review the clinical manifestations, pathophysiology, and oncologic implications of the major mucocutaneous paraneoplastic syndromes that can appear in patients with hematologic malignancies. METHODS: A comprehensive search of the medical literature was conducted. RESULTS: In vesiculobullous conditions, although the primary lesions are blisters, observed abnormalities may include large, flaccid bullae (pemphigus vulgaris), superficial crusted erosions (pemphigus foliaceus), or erythema multiforme-like lesions (paraneoplastic pemphigus). Paraneoplastic neutrophilic dermatoses include Sweet's syndrome and pyoderma gangrenosum. In both of these conditions, the skin lesions are characterized by a dermal infiltrate of mature neutrophils. Vascular dermatoses include vasculitis and erythromelagia. Papulosquamous conditions are characterized by small (papules) or large (plaques) raised skin lesions and are usually associated with solid tumors. Amyloidosis is a malignancy-related condition that probably stems from immune dysregulation. RECOMMENDATIONS: Continued surveillance of patients with potential cutaneous paraneoplastic syndromes is necessary, since the malignancy may not be immediately detectable. Some of the cutaneous paraneoplastic syndromes will respond to specific measures, such as systemic corticosteroid therapy, but for the most part, successful resolution requires eradication of the underlying malignancy.

Hematologic Diseases↗
PubMed1989

Induction of acantholysis in organ explant culture by penicillamine and captopril.

Pemphigus is an autoimmune disease proved to be mediated by IgG autoantibodies. Skin lesions clinically and histologically identical to pemphigus may occur in patients receiving penicillamine and captopril, but some of these patients lack circulating or tissue-bound autoantibodies. Therefore, we examined the ability of these drugs to produce acantholysis directly in organ explant culture. Human skin explants were prepared from split-thickness graft skin from adults and from neonatal foreskins. Explants were cultured in media containing 0.1 to 200 mmol/L of penicillamine or captopril; parallel drug-free control cultures were also prepared. Acantholysis occurred in all split-thickness graft skin cultures incubated for 72 hours with at least 20 mmol/L of penicillamine and at 24 to 48 hours in those incubated with at least 10 mmol/L of captopril. Acantholysis occurred less frequently in foreskin cultures, being present in 1 (8%) of 12 of those exposed to at least 20 mmol/L of penicillamine and 3 (12%) of 25 of those exposed to at least 10 mmol/L of captopril. None of the parallel drug-free control cultures developed acantholysis. Subcorneal acantholysis, resembling that seen in pemphigus foliaceus, and suprabasilar acantholysis, resembling that seen in pemphigus vulgaris, were induced in vitro. Our results indicate that both drugs can act as ligands and produce acantholysis in organ explant culture in the absence of autoantibody. This ligand-induced acantholysis may also be responsible for induction of the disease in vivo in those patients who lack demonstrable autoantibodies.

Acantholysis↗
PubMed1994

[Molecular diversity of desmosomal cadherins and their potential as markers in the histodiagnosis of carcinomas].

Desmosomal cadherins, transmembrane glycoproteins of the cadherin family of cell adhesion molecules, comprise two subfamilies, the desmogleins and the desmocollins, each of which consist of at least 3 distinct proteins encoded by individual genes. We have analyzed the expression of desmogleins Dsg1 (Pemphigus foliaceus antigen), Dsg2 ("simple epithelium type") and Dsg3 (Pemphigus vulgaris antigen) in various normal tissues and diverse carcinomas, using RNase protection assays. We found that the gene encoding Dsg2 was expressed in most epithelial tissues and carcinomas whereas mRNAs encoding Dsg1 and Dsg3 were primarily restricted to stratified squamous epithelia and certain (mostly squamous cell) carcinomas. Antisera raised against recombinant polypeptides corresponding to various parts of Dsg2 produced immunostaining along intercellular borders of simple epithelia and basal cells of non-cornifying stratified squamous epithelia but were negative with most cells of epidermis. Preliminary analyses of carcinomas revealed comparable patterns. These cell type-specific differences in the molecular composition of desmosomes, which are also reflected in carcinomas, open new possibilities for the histological classification and subtyping of carcinomas. Moreover, the functional importance of desmosomal cadherins in the adhesion of carcinoma cells and during metastasis makes them a promising marker system for the assessment of the biological behaviour of carcinomas.

Biomarkers, Tumor↗
PubMed1999

Desmoglein 1 and desmoglein 3 are the target autoantigens in herpetiform pemphigus.

OBJECTIVE: To determine the cell surface autoimmune target of herpetiform pemphigus (HP). DESIGN: Serum samples of HP were examined by immunoblot studies with human epidermal extracts, enzyme-linked immunosorbent assay with baculovirus-expressed recombinant desmoglein (rDsg) 1 and rDsg3, and immunoadsorption assay with rDsg. PATIENTS: Twenty serum samples were obtained from patients with HP who have typical clinical and histological features. All serum samples showed positive staining against keratinocyte cell surfaces by indirect immunofluorescence studies with healthy human skin. RESULTS: Immunoblot results showed that of 17 HP serum samples, only 5 reacted with a 160-kd band and 1 reacted with a 130-kd band. Results of enzyme-linked immunosorbent assays with rDsg1 and rDsg3 demonstrated that of 20 HP serum samples, 16 were positive against Dsg1 and 4 were positive against Dsg3. No serum samples reacted with both. Furthermore, in 19 of 20 HP serum samples, immunoreactivity against keratinocyte cell surfaces was completely removed by preincubation with rDsg1 and rDsg3 as shown by indirect immunofluorescence, excluding a possibility that these HP sera contain autoantibodies against other cell surface molecules. CONCLUSIONS: Dsg1 and Dsg3 are the major cell surface target molecules of HP, suggesting that most cases of HP are clinical variants of pemphigus foliaceus and that the rest might be variants of pemphigus vulgaris.

Autoantigens↗
PubMed1997

Sensitivity of indirect immunofluorescence, substrate specificity, and immunoblotting in the diagnosis of pemphigus.

BACKGROUND: Several assays are available to detect pemphigus antibodies. The most commonly used are indirect immunofluorescence (IIF) and immunoblotting (IB). OBJECTIVE: Our purpose was to compare the sensitivity of these assays in detecting pemphigus antibodies. METHODS: Fifty-two sera from 41 patients with pemphigus vulgaris (PV) and 22 sera from 18 patients with pemphigus foliaceus (PF) were tested concurrently for the presence of pemphigus antibodies. The IIF studies were conducted with two different substrates: monkey and guinea pig esophagus. RESULTS: Pemphigus antibodies were detected with equal sensitivity by IIF in patients with PV and PF (i.e., positive in 87% and 86% of sera, respectively). By contrast, IB assay was much less sensitive in PF than in PV (i.e., positive in 45% vs 83% of sera, respectively). The antibodies in PV generally reacted more strongly against monkey esophagus, whereas those in PF reacted more strongly against guinea pig esophagus. All patients with intercellular antibodies that reacted more strongly against monkey than guinea pig esophagus had PV, whereas all those with intercellular antibodies that reacted more strongly against guinea pig than monkey esophagus had PF. CONCLUSION: IIF is a more sensitive assay than IB for detecting antibodies associated with PF. The substrate specificity of the antibodies provides a simple means to distinguish between PV and PF.

Antibodies↗
PubMedDate not supplied

Study of desmoglein 1 and 3 antibody levels in relation to disease severity in Indian patients with pemphigus.

OBJECTIVES: To conduct a cross-sectional study to compare Dsg1 and Dsg3 antibody levels independently with severity of disease activity in pemphigus vulgaris (PV) and pemphigus foliaceus (PF). METHODS: Blood samples from 44 patients with pemphigus (PV-38, PF-6) were analyzed using ELISA. The severity of skin and mucosal disease was graded using a score from 0 to 3. RESULTS: A statistically significant correlation between increase in Dsg 3 antibody titres with severity of oral involvement and Dsg 1 titres with severity of skin involvement was found in both PV and PF patients (p < 0.01). However, we were unable to demonstrate a relationship between increased titres of Dsg1 and Dsg 3 antibodies with oral and skin involvement respectively. CONCLUSION: This study suggests that the severity of skin and oral disease in pemphigus is determined by the quantities of Dsg1 and Dsg3 antibodies respectively.

Adolescent↗