Klinik und immunpathologie bei 48 patienten mit pemphigus

Translated title of the contribution: Clinical and immunpathological findings in 48 patients with pemphigus

K. Hahn, W. Kippes, Masayuki Amagai, B. Rzany, E. B. Bröcker, D. Zillikens

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

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 immunpathological findings in all pemphigus patients who were diagnosed in the Department of Dermatology at the University of Wurzburg over the past 10 years. Patients/Methods. Based on a retrospective study, clinical and immunpathological findings in 48 patients with pemphigus were recorded. All patients had positive findings by direct and/or indirect immunfluorescence 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 immunfluorescence 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 immunfluorescence 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.

Original languageGerman
Pages (from-to)670-677
Number of pages8
JournalHautarzt
Volume51
Issue number9
DOIs
Publication statusPublished - 2000

Fingerprint

Pemphigus
Desmoglein 3
Desmoglein 1
Microscopy
Autoantibodies
Skin
Mucous Membrane
Immunoglobulin G
Serum

Keywords

  • ELISA
  • Epidemiology
  • Immunfluorescence
  • Pemphigus

ASJC Scopus subject areas

  • Dermatology

Cite this

Hahn, K., Kippes, W., Amagai, M., Rzany, B., Bröcker, E. B., & Zillikens, D. (2000). Klinik und immunpathologie bei 48 patienten mit pemphigus. Hautarzt, 51(9), 670-677. https://doi.org/10.1007/s001050051193

Klinik und immunpathologie bei 48 patienten mit pemphigus. / Hahn, K.; Kippes, W.; Amagai, Masayuki; Rzany, B.; Bröcker, E. B.; Zillikens, D.

In: Hautarzt, Vol. 51, No. 9, 2000, p. 670-677.

Research output: Contribution to journalArticle

Hahn, K, Kippes, W, Amagai, M, Rzany, B, Bröcker, EB & Zillikens, D 2000, 'Klinik und immunpathologie bei 48 patienten mit pemphigus', Hautarzt, vol. 51, no. 9, pp. 670-677. https://doi.org/10.1007/s001050051193
Hahn K, Kippes W, Amagai M, Rzany B, Bröcker EB, Zillikens D. Klinik und immunpathologie bei 48 patienten mit pemphigus. Hautarzt. 2000;51(9):670-677. https://doi.org/10.1007/s001050051193
Hahn, K. ; Kippes, W. ; Amagai, Masayuki ; Rzany, B. ; Bröcker, E. B. ; Zillikens, D. / Klinik und immunpathologie bei 48 patienten mit pemphigus. In: Hautarzt. 2000 ; Vol. 51, No. 9. pp. 670-677.
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abstract = "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 immunpathological findings in all pemphigus patients who were diagnosed in the Department of Dermatology at the University of Wurzburg over the past 10 years. Patients/Methods. Based on a retrospective study, clinical and immunpathological findings in 48 patients with pemphigus were recorded. All patients had positive findings by direct and/or indirect immunfluorescence 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 immunfluorescence 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 immunfluorescence 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.",
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AB - 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 immunpathological findings in all pemphigus patients who were diagnosed in the Department of Dermatology at the University of Wurzburg over the past 10 years. Patients/Methods. Based on a retrospective study, clinical and immunpathological findings in 48 patients with pemphigus were recorded. All patients had positive findings by direct and/or indirect immunfluorescence 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 immunfluorescence 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 immunfluorescence 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.

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