TY - JOUR
T1 - A unique form of endemic pemphigus in northern Colombia
AU - Abrèu-Velez, Ana María
AU - Hashimoto, Takashi
AU - Bollag, Wendy B.
AU - Arroyave, Sergio Tobón
AU - Abrèu-Velez, Clara Eugenia
AU - Londoño, Martha Luz
AU - Montoya, Fernando
AU - Beutner, Ernst H.
N1 - Funding Information:
We acknowledge Stella Prada de C, MD, Walter Leon Herrera, MD, Maria Mercedes Yepes, MD, Juan Guillermo Maldonado, MD, Jorge Botero, MD, Edinson Villa, Fredy Vélez, MD, Juan Esteban Arroyave, MD, Alex Pabón, MD, Armando Muñoz, MD, Andres Jaramillo, PhD, Doris Ruiz, and all co-workers that participated in the visits to El Bagre for their contribution to this research and humanitarian program, as well as all those who donated goods for the patients. We also appreciate those who assisted the endemic PF patients at the Hospital Universitario San Vicente de Paul in Medellín. We extend our thanks to the El Bagre community, to Hospital Nuestra Señora de El Bagre, and to the Major Office and Health Jurisdiction in El Bagre. We also thank Darius Mehregan, MD, of Pinkus Laboratories for the study of skin biopsy specimens and for the micrographs used in this study. Special thanks go to Marcos Restrepo, MD, at ICMT for support; to Richard Plunkett, MD, and Janet Zilliox of Beutner Laboratories for testing specimens of endemic PF cases and for reading and commenting on this manuscript; and to Dawn Martin for finalizing the manuscript. This article represents a portion of the PhD thesis of Ana María Abréu Velez, who received a scholarship from Colciencias, Colombia, administered by the Latin American Scholarship Program of American Universities (USA).
Funding Information:
Funding sources: Grants from the Embassy of Japan in Colombia; Mineros de Antioquia, S.A.; Instituto Colombiano de Medicina Tropical as part of the Instituto de Ciencias de la Salud; Universidad de Antioquia; Direccion Seccional de Salud de Antioquia; and Medical College of Georgia and the National Institutes of Health (grant no. AR45212). Dr Abrèu-Velez received a scholarship from Colciencias, Colombia. Studies at Beutner Laboratories on monkey esophagus sections were aided by Beutner Laboratories and by National Institutes of Health grant No. RR00163 to the Oregon Primate Center and by Beutner Laboratories.
PY - 2003/10/1
Y1 - 2003/10/1
N2 - Background: Endemic forms of pemphigus are a unique group of autoimmune diseases that represent opportunities to study interactions of the environment and genetics with the immune system. The restriction to relatively well-defined regions of South and Central America and perhaps Africa characterizes these diseases. Objectives: The aims of this study were to confirm the endemic nature of a new type of autoimmune disease occurring in a mining town in northeastern Colombia in the El Bagre area, to characterize it, and to compare it with other forms of endemic pemphigus. Methods: A 10-year prospective, controlled epidemiologic, humanitarian, and immunologic fieldwork case-control survey was performed in El Bagre, Colombia. Results: Our work revealed that this disease is endemic in rural areas surrounding El Bagre. The disease appeared in 4.7% of middle-aged and older men and postmenopausal women from these rural areas. This disease differs from previously described forms of endemic pemphigus. It shares some heterogeneous immunoreactivity with paraneoplastic pemphigus but is not associated with malignant tumors. The disease resembles Senear-Usher syndrome (pemphigus and lupus) but occurs endemically either with a localized stable clinical course or in a systemic form. This systemic form may affect organs other than skin and is characterized by episodic relapses and poor prognosis in comparison with the localized form. Conclusion: We have confirmed endemic pemphigus foliaceus in El Bagre as an autoimmune disease that shares features with Senear-Usher syndrome but occurs in an endemic fashion. Heterogeneous antigenic reactivity is observed as in paraneoplastic pemphigus but with no evidence of association with neoplasia. In addition, constant exogenous antigenic stimulation and a genetic predisposition may be required in the pathogenesis of this disease.
AB - Background: Endemic forms of pemphigus are a unique group of autoimmune diseases that represent opportunities to study interactions of the environment and genetics with the immune system. The restriction to relatively well-defined regions of South and Central America and perhaps Africa characterizes these diseases. Objectives: The aims of this study were to confirm the endemic nature of a new type of autoimmune disease occurring in a mining town in northeastern Colombia in the El Bagre area, to characterize it, and to compare it with other forms of endemic pemphigus. Methods: A 10-year prospective, controlled epidemiologic, humanitarian, and immunologic fieldwork case-control survey was performed in El Bagre, Colombia. Results: Our work revealed that this disease is endemic in rural areas surrounding El Bagre. The disease appeared in 4.7% of middle-aged and older men and postmenopausal women from these rural areas. This disease differs from previously described forms of endemic pemphigus. It shares some heterogeneous immunoreactivity with paraneoplastic pemphigus but is not associated with malignant tumors. The disease resembles Senear-Usher syndrome (pemphigus and lupus) but occurs endemically either with a localized stable clinical course or in a systemic form. This systemic form may affect organs other than skin and is characterized by episodic relapses and poor prognosis in comparison with the localized form. Conclusion: We have confirmed endemic pemphigus foliaceus in El Bagre as an autoimmune disease that shares features with Senear-Usher syndrome but occurs in an endemic fashion. Heterogeneous antigenic reactivity is observed as in paraneoplastic pemphigus but with no evidence of association with neoplasia. In addition, constant exogenous antigenic stimulation and a genetic predisposition may be required in the pathogenesis of this disease.
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U2 - 10.1067/S0190-9622(03)00851-X
DO - 10.1067/S0190-9622(03)00851-X
M3 - Article
C2 - 14512903
AN - SCOPUS:18244432336
SN - 0190-9622
VL - 49
SP - 599
EP - 608
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 4
ER -