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W Kippes

Publications and source records attributed to W Kippes.

3 recordsLinked to original sources

[Clinical aspects and immunopathology in 48 patients with pemphigus].

BACKGROUND AND OBJECTIVE: Pemphigus is a rare intraepidermal autoimmune bullous disease. Two major variants, pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are distinguished. The aim of this study was to document the clinical and immunopathological findings in all pemphigus patients who were diagnosed in the Department of Dermatology at the University of Würzburg over the past 10 years. PATIENTS/METHODS: Based on a retrospective study, clinical and immunopathological findings in 48 patients with pemphigus were recorded. All patients had positive findings by direct and/or indirect immunofluorescence microscopy. RESULTS: Between January 1989 and August 1998, 48 patients were diagnosed with pemphigus at our institution; 31 patients had PV and 17 PF. The average age (+/- standard deviation) of PV patients was 55 (+/- 17) and of PF patients 60 (+/- 12) years. All PV patients showed involvement of mucous membranes and in 65% of cases, the skin was also involved. In contrast, PF patients had involvement only of the skin. By direct immunofluorescence microscopy, intercellular deposits of IgG and C3 were detected in 89% and 78% of PV cases, respectively. In PF, intercellular deposits of IgG were found in 94% and of C3 in 75% of cases. By indirect immunofluorescence microscopy on monkey esophagus, 94% of the PV and 88% of the PF patients revealed circulating serum antibodies. In 30 patients, we characterized the immune response by ELISA using recombinant desmoglein 1 and 3. All PF sera showed autoantibodies against desmoglein 1 and all PV sera against desmoglein 3. In PV with both mucous membrane and skin involvement, antibodies to both desmoglein 3 and 1 were detected. CONCLUSIONS: Our results confirm the correlation of the autoantibody profile with the clinical phenotype of pemphigus.

Adult↗

[Immunopathologic changes in 115 patients with bullous pemphigoid].

Bullous pemphigoid (BP) is an autoimmune bullous disease of the elderly. Patients' auto-antibodies are directed against BP180, a transmembrane hemidesmosomal glycoprotein of basal keratinocytes. Between 1989 and 1998, we identified 115 patients with BP in the Department of Dermatology at the University of Würzburg. This is one of the largest groups of patients with BP studied to date. In all patients, direct and/or indirect immunofluorescence microscopy studies were positive. The average age (+/- standard deviation) of the patients was 75 +/- 12 years, the median was 78 years. The youngest patient was 39 years of age; the oldest, 99 years. 54% of the patients were female and 46% male. In 24% of the patients, an involvement of oral, and in 7% of genital mucous membranes was seen. 98% of the patients complained about pruritus. By direct immunofluorescence microscopy, linear deposits of C3 and/or IgG were detected at the basement membrane zone of perilesional skin biopsies. By indirect immunofluorescence microscopy using NaCl-separated human skin, 87% of cases revealed circulating serum antibodies whereas on monkey esophagus, only 72% of sera were found positive. In 85% of the patients, we detected elevated serum levels of total IgE before the initiation of treatment. In those sera that were analyzed for reactivity with BP180, autoantibodies to the immunodominant NC16 A domain of BP180 were detected in 89% by immunoblot analysis and in 93% by ELISA. Therefore, in most patients, BP can be diagnosed serologically.

Aged↗

Increased risk of bullous pemphigoid in male and very old patients: A population-based study on incidence.

No data are available on gender- and age-specific incidences for bullous pemphigoid (BP). The aim of this study was to calculate incidences for different gender- and age-strata and to assess risk differences between these strata in BP. A retrospective population-based cohort was recruited from all patients diagnosed with BP in 2 well-defined regions of Germany. The average population number was 1.7 million, and the observation period was 9 years (1989-1997). Incidences were calculated as newly diagnosed cases for a population of 1 million per year. Confidence intervals (CI) were estimated based on a Poisson distribution. For evaluation of risk between different age and gender strata a Poisson regression analysis was used. The highest incidence was calculated for individuals older than 90 years, with 398 (CI: 360, 439) new cases of BP per 1 million residents for men, and 87 (CI: 70, 108) new cases per 1 million residents for women. Risk (95% CI) was 1.9 (1.3, 2.9) fold higher in men than in women. In addition, the risk for BP was increased for patients above the age of 60 years. The highest risk was found for patients older than 90 years. For this age group, the risk was 297 (CI: 107, 826) fold higher than in patients 60 years of age and younger. Men are affected by BP almost twice as often as women. The risk for BP increases rapidly beyond the age of 60 years. Because the structure of the European population is shifting towards the aged, more people are expected to suffer from BP in the coming decades.

Age Factors↗