A unique form of endemic pemphigus in northern Colombia

Ana María Abrèu-Velez, Takashi Hashimoto, Wendy B Bollag, Sergio Tobón Arroyave, Clara Eugenia Abrèu-Velez, Martha Luz Londoño, Fernando Montoya, Ernst H. Beutner

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)599-608
Number of pages10
JournalJournal of the American Academy of Dermatology
Volume49
Issue number4
DOIs
StatePublished - Oct 1 2003

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Pemphigus
Colombia
Usher Syndromes
Autoimmune Diseases
Central America
Endemic Diseases
South America
antineoplaston A10
Genetic Predisposition to Disease
Immune System
Neoplasms
Recurrence
Skin

ASJC Scopus subject areas

  • Dermatology

Cite this

Abrèu-Velez, A. M., Hashimoto, T., Bollag, W. B., Arroyave, S. T., Abrèu-Velez, C. E., Londoño, M. L., ... Beutner, E. H. (2003). A unique form of endemic pemphigus in northern Colombia. Journal of the American Academy of Dermatology, 49(4), 599-608. https://doi.org/10.1067/S0190-9622(03)00851-X

A unique form of endemic pemphigus in northern Colombia. / Abrèu-Velez, Ana María; Hashimoto, Takashi; Bollag, Wendy B; Arroyave, Sergio Tobón; Abrèu-Velez, Clara Eugenia; Londoño, Martha Luz; Montoya, Fernando; Beutner, Ernst H.

In: Journal of the American Academy of Dermatology, Vol. 49, No. 4, 01.10.2003, p. 599-608.

Research output: Contribution to journalArticle

Abrèu-Velez, AM, Hashimoto, T, Bollag, WB, Arroyave, ST, Abrèu-Velez, CE, Londoño, ML, Montoya, F & Beutner, EH 2003, 'A unique form of endemic pemphigus in northern Colombia', Journal of the American Academy of Dermatology, vol. 49, no. 4, pp. 599-608. https://doi.org/10.1067/S0190-9622(03)00851-X
Abrèu-Velez AM, Hashimoto T, Bollag WB, Arroyave ST, Abrèu-Velez CE, Londoño ML et al. A unique form of endemic pemphigus in northern Colombia. Journal of the American Academy of Dermatology. 2003 Oct 1;49(4):599-608. https://doi.org/10.1067/S0190-9622(03)00851-X
Abrèu-Velez, Ana María ; Hashimoto, Takashi ; Bollag, Wendy B ; Arroyave, Sergio Tobón ; Abrèu-Velez, Clara Eugenia ; Londoño, Martha Luz ; Montoya, Fernando ; Beutner, Ernst H. / A unique form of endemic pemphigus in northern Colombia. In: Journal of the American Academy of Dermatology. 2003 ; Vol. 49, No. 4. pp. 599-608.
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abstract = "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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