Endemic pemphigus vulgaris.
BACKGROUND
Investigators from Brasilia, Brazil, observed several patients with a mucocutaneous disease that resembles pemphigus vulgaris clinically and histologically but with epidemiological features of fogo selvagem. Our objective was to characterize antidesmoglein 3 and antidesmoglein 1 autoantibody profiles in these unique patients who reside in Goiânia and Brasilia, Brazil, known endemic regions of fogo selvagem.


METHODS
We performed serological evaluation of 8 patients with a mucocutaneous disease clinically and histologically consistent with pemphigus vulgaris, as well as 27 healthy relatives of patients with fogo selvagem who reside in these endemic areas. Serum samples from all 8 patients bound desmoglein 3 by cold immunoprecipitation and from 6 patients by enzyme-linked immunosorbent assay, while serum samples from 4 patients bound desmoglein 1 by cold immunoprecipitation and by enzyme-linked immunosorbent assay. Antidesmoglein 3 autoantibodies were detected in 4 of 27 healthy donors by cold immunoprecipitation and by enzyme-linked immunosorbent assay, whereas antidesmoglein 1 autoantibodies were detected in 6 individuals by cold immunoprecipitation and in 3 individuals by enzyme-linked immunosorbent assay.


CONCLUSIONS
These findings provide serological evidence of a new endemic variant of pemphigus vulgaris.
Rocha-Alvarez
Rosicler
R
Department of Dermatology, Universidade Federal de Brasilia, Brasilia, Distrito Federal.
Ortega-Loayza
Alex G
AG
Friedman
Horacio
H
Campbell
Iphis
I
Aoki
Valeria
V
Rivitti
Evandro A
EA
Dasher
David
D
Li
Ning
N
Diaz
Luis A
LA
D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D001938:Brazil / Q000453:epidemiology; D016022:Case-Control Studies; D051183:Desmoglein 1 / Q000276:immunology; D051184:Desmoglein 3 / Q000276:immunology; D019353:Endemic Diseases; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000097:blood / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology; D016036:Seroepidemiologic Studies
D001323:Autoantibodies; D051183:Desmoglein 1; D051184:Desmoglein 3