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Endemic Pemphigus Vulgaris
Rosicler Rocha-Alvarez, MD;
Alex G. Ortega-Loayza, MD;
Horacio Friedman, MD;
Iphis Campbell, MD;
Valeria Aoki, MD;
Evandro A. Rivitti, MD;
David Dasher, MD;
Ning Li, PhD;
Luis A. Diaz, MD; for the Cooperative Group on Fogo Selvagem Research
Arch Dermatol. 2007;143(7):895-899.
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.
Observations 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.
Conclusion These findings provide serological evidence of a new endemic variant of pemphigus vulgaris.
Author Affiliations: Department of Dermatology, Universidade Federal de Brasilia, Brasilia, Distrito Federal (Drs Rocha-Alvarez, Friedman, and Campbell), and Department of Dermatology, Universade de São Paulo, São Paulo (Drs Aoki and Rivitti), Brazil; and Department of Dermatology, University of North Carolina at Chapel Hill (Drs Ortega-Loayza, Dasher, Li, and Diaz).
Group Information: A list of the members of the Cooperative Group on Fogo Selvagem Research has been published in J Invest Dermatol. 1989;92(1):4-12 and J Am Acad Dermatol. 1989;20(4):657-669.
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