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


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.
Hahn
K
K
Klinik und Poliklinik für Haut- und Geschlechtskrankheiten, Universität Würzburg.
Kippes
W
W
Amagai
M
M
Rzany
B
B
Bröcker
E B
EB
Zillikens
D
D
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D001706:Biopsy; D015820:Cadherins / Q000276:immunology; D003176:Complement C3 / Q000378:metabolism; D051183:Desmoglein 1; D051184:Desmoglein 3; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007105:Immune Complex Diseases / Q000175:diagnosis / Q000473:pathology; D007074:Immunoglobulin G / Q000378:metabolism; D008297:Male; D008856:Microscopy, Fluorescence; D008875:Middle Aged; D009061:Mouth Mucosa / Q000473:pathology; D010392:Pemphigus / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology
D001323:Autoantibodies; D015820:Cadherins; D003176:Complement C3; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3; D007074:Immunoglobulin G