Pemphigus, analysis of 155 patients.

BACKGROUND: Pemphigus vulgaris is a chronic autoimmune mucocutaneous blistering disease. In the last 20 years, immunomodulatory agents have been added to the therapeutic armamentarium. Only few recent studies have evaluated the long-term outcome of pemphigus and possible prognostic parameters in a large group of patients. The aim of this study was to evaluate and analyse the course and prognostic factors of pemphigus in patients followed from 1976 to 2004. PATIENTS AND METHODS: The study group consisted of 155 patients attending the pemphigus clinic of a major tertiary-care medical centre. Background, clinical and treatment data were derived from the patient files and telephone contact. Statistical analysis was performed with Pearson correlation, Fisher exact test, and univariate and multivariate logistic regression models. RESULTS: Age < 40 years at disease onset, Sephardic Jewish origin, and mucosal involvement at diagnosis and high number of relapses were found to be independent prognostic factors of poor outcome. A long (> 1 year) primary remission was a good prognostic factor. During the 26-year study period, 16 patients died. None of the deaths was directly related to either the disease or a complication of treatment. CONCLUSIONS: The course and outcome of pemphigus are worse in patients who are young at diagnosis (< 40 years) or of Sephardic Jewish origin. Mucosal involvement at diagnosis and poor response to treatment also predict poor outcome. The mortality rate of pemphigus is apparently lower than reported in the literature, perhaps because of the contemporary use of adjuvant immunomodulatory therapeutic agents.

Pemphigus--analysis of epidemiological factors in 155 patients.

BACKGROUND: Pemphigus vulgaris is a chronic autoimmune mucocutaneous blistering disease. Only a few studies have evaluated the epidemiological and aetiological parameters of pemphigus vulgaris in a large group of patients over the long term. METHODS: The sample included 155 patients with a diagnosis of pemphigus who attended the pemphigus clinic of a major tertiary medical centre from 1976 to 2004. Data were obtained from the patient files and entered into an ad hoc form; patients were contacted by telephone for missing information. RESULTS: The female-to-male ratio was 1.5 : 1. Non-Ashkenazi Jews accounted for 37% of the sample. In only 10% of the patients was a potential aetiologic or precipitating factor identified. CONCLUSIONS: Pemphigus vulgaris is characterized by a female predominance, consistent with other autoimmune disease. The gender, age and ethnic distribution of affected patients have not changed in the last 40 years. In the vast majority of cases, the aetiologic or precipitating factor is unknown, although drugs appear to be very rare.

Flow cytometric DNA analysis of classic and steroid-induced Kaposi's sarcoma.

Flow cytometric DNA analysis of various tumours has indicated a correlation between the degree of malignancy and ploidy; results which could have clinical significance. We analysed the ploidy of Kaposi's sarcoma (KS) tumours, and classified the results according to clinical history and histological findings. We found that patients on steroid treatment had an aneuploid pattern, and most of the patients with classic-type KS had a diploid pattern on flow cytometry.

The efficacy of combined treatment with prednisone and cyclosporine in patients with pemphigus: preliminary study.

BACKGROUND: Cyclosporine, a potent immunosuppressive drug, has been suggested to be beneficial in the treatment of some immune-mediated dermatoses, including pemphigus. OBJECTIVE: The aim of the present study was to evaluate the efficacy and safety of combined treatment with prednisone and cyclosporine compared with prednisone alone in patients with pemphigus. METHODS: Sixteen hospitalized patients with pemphigus vulgaris received combined treatment with cyclosporine and prednisone for 12 months. Cyclosporine and prednisone were given orally at an initial dose of 5 mg/kg/day and 60 to 80 mg/day, respectively. The cyclosporine dose was adjusted to obtain plasma levels of 100 to 150 ng/L. A historical control group was composed of 15 patients with pemphigus who received prednisone at an initial dose of 120 mg/day, which was decreased according to clinical response. The clinical response, serum titer of autoantibodies, and frequency of side effects during a 1-year follow-up period were evaluated and compared. RESULTS: The 16 patients in the combined therapy group achieved clinical remission within 25 days or less, a shorter period than for the control group, although the difference was not statistically significant. New blister formation ceased after a mean of 11.1 +/- 7.9 days of onset of treatment in the combined treatment group versus 20.5 +/- 12 days in the control group (p = 0.004). Hospital stay was shorter in the combined treatment group (mean, 32.6 +/- 12.5 days) than in the control group (mean, 50.7 +/- 17.1 days; p = 0.003).(ABSTRACT TRUNCATED AT 250 WORDS)

[A pustular form of loxoscelism (bite of the brown recluse spider)]. / Pustulöse Form des Loxoszelismus (Biss der braunen Einsiedlerspinne).

We report on a 42-year-old man who showed the typical clinical picture after being bitten by a brown recluse spider (Loxosceles reclusa). The patient developed a pustular eruption during the acute phase of the disease. To the best of our knowledge, this is the first report on pustular loxoscelism.

Familial pemphigus vulgaris.

Familial pemphigus vulgaris was found in a Jewish woman and her son who developed the disease within a period of 18 months from one another. HLA typing was performed in the mother and son as well as in the unaffected father and sister. Examination of the HLA antigens in this family indicates that there may be different variants of DR4, DQw3 and that one of them carried by the mother and son as part of the haplotype A30, B18, DR4, DQw3 is associated with pemphigus vulgaris. Our findings are in favor of the concept that particular polymorphic residues of class II major histocompatibility molecules are correlated with disease susceptibility.

Familial lichen planus.

Familial lichen planus (FLP) was observed to have developed within a period of 3 years in 2 sisters as well as in the son of one of the women. In contrast to typical FLP, the eruption was generalized in only 1 of these patients; no atypical forms were observed; the response to topical treatment with steroids was rapid, and the relapses were few and mild. Previous reports of familial cases of lichen planus as well as the long interval between onset of the disease in the affected members of the family speak in favor of a genetic predisposition. HLA typing revealed HLA DR in all 3 patients. There was no increased incidence of HLA B7, HLA A3 or HLA A28.

Determination of IgG subclasses in patients with pemphigus with active disease and in remission.

IgG subclasses were determined in perilesional skin of 13 patients with pemphigus with active disease and of 14 patients in a state of clinical remission. Using indirect immunofluorescence technique, frozen sections were incubated with mouse monoclonal antihuman IgG1, IgG2, IgG3, and IgG4 followed by a second incubation with fluorescein isothiocyanate-conjugated goat antimouse IgG. The results showed that among patients with active disease, IgG1 was found in all of them and IgG4 in 85%, while IgG2 and IgG3 were found in 54% and 77%, respectively. For patients in remission the most common subclass was IgG4 in 79% of patients, and in a decreased order IgG1, 50%; IgG3, 29%; and IgG2, 14%. It appears that IgG1 and IgG4 are predominant among patients with active lesions. IgG1 seems to be the most sensitive indicator for activity of the disease. IgG4, normally found in the lowest concentration in human serum, is the most common subclass in patients who are in remission. IgG3 and C3 may have a predictive value for remission.