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Updated: May 22, 2026
12:23
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Endemic pemphigus over a century: Part II
Ana María Abréu-Vélez1, Ana Maria Roselino, Michael S Howard
1Georgia Dermatopathology Associates, Atlanta, Georgia, USA.
North American Journal of Medical Sciences
|May 25, 2012
Summary
Endemic pemphigus foliaceus (EPF) autoantigens vary geographically, with desmogleins implicated in most cases. Further research is needed to fully understand EPF
Area of Science:
- Dermatology
- Immunology
- Genetics
Background:
- Endemic pemphigus foliaceus (EPF) is an autoimmune blistering disease with distinct geographic foci.
- EPF predominantly affects young Amerindians in Central and South America, with specific variants in Colombia and Tunisia.
- Demographic variations exist, including age and sex predilections in different EPF foci.
Purpose of the Study:
- To review and synthesize current knowledge on autoantigens in EPF.
- To summarize immunologic and genetic findings across various EPF populations.
- To compare the characteristics of different geographic variants of EPF.
Main Methods:
- Retrospective literature review.
- Compilation and comparison of data from multiple geographic foci of EPF.
- Analysis of reported autoantigens and immunologic/genetic studies.
Main Results:
- Desmogleins are primary autoantigens in Tunisian and most American EPF cases.
- El Bagre-EPF involves plakins and desmogleins.
- Antibody-mediated immune response is the principal pathophysiology in all EPF variants.
Conclusions:
- Genetic susceptibility likely plays a role in EPF development.
- Specific causative genes for EPF remain unconfirmed.
- Further immunological and genetic investigations are essential to fully characterize EPF.
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