Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus

Ana Maria Abreu-Velez; Yulieth A. Upegui-Zapata; Carlos A. Valencia-Yepes; Eduardo Upegui-Quiceno; Alejandra M. Jiménez-Echavarría; César D. Niño-Pulido; Bruce R. Smoller; Michael S. Howard

doi:10.5826/dpc.0903a02

Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus

Authors

Ana Maria Abreu-Velez 4048764897 http://orcid.org/0000-0002-7692-4133
Yulieth A. Upegui-Zapata PECET Group, Pharmaceutical Sciences, Medical Research Institute, School of Medicine, University of Antioquia, Medellín, Colombia
Carlos A. Valencia-Yepes Department of Education, University of Antioquia, Medellín, Colombia
Eduardo Upegui-Quiceno PECET Group, University of Antioquia, Medellín, Colombia
Alejandra M. Jiménez-Echavarría PECET Group, University of Antioquia, Medellín, Colombia
César D. Niño-Pulido CES University, Medellín, Colombia
Bruce R. Smoller Departments of Pathology and Laboratory Medicine & Dermatology, University of Rochester Medical Center, School of Medicine and Dentistry, Rochester, NY, USA
Michael S. Howard Georgia Dermatopathology Associates, Atlanta, GA, USA

Keywords:

areae compositae of the heart, endemic pemphigus foliaceus, MYZAP, left cardiac hypertrophy, p0071, ARVCF, desmoplakins I and II

Abstract

Background: A new variant of endemic pemphigus foliaceus in El Bagre (El Bagre-EPF), Colombia, South America, shares features with Senear-Usher syndrome and occurs in an endemic fashion. Patients affected by El Bagre-EPF have heterogeneous antigenic reactivity not only to the skin but to other organs, including the heart. Here we test for autoantibodies to the areae compositaeof the heart (structure consisting of typical desmosomal amalgamated fascia adherensmolecules)and evaluate any possible clinical correlation.

Methods: A case-control study comparing 45 patients and 45 controls from the endemic area, matched by demographics including age, gender, weight, work activities, and comorbidities, was performed. Direct and indirect immunofluorescence, immunohistochemistry, confocal microscopic studies, and echocardiogram studies were completed.

Results: The main clinical abnormally seen in the El Bagre-EPF patients was left ventricular hypertrophy in 15/45 patients, compared with no such findings in the control population (P < 0.1). Seventy percent of El Bagre-EPF patients and none of the controls displayed polyclonal autoreactivity using different immunoglobulins and complement to the areae compositae of the heart using different methods and antibodies (P < 0.1).

Conclusions: Patients affected by El Bagre-EPF demonstrated autoantibodies to the areae compositae of the heart. This finding was associated with left ventricular hypertrophic cardiomyopathy.The areae compositaemay play a role incell junction tension and the El Bagre-EPF patients’ autoantibodies possibly disrupting these junctions and thereby contributing to the left ventricular hypertrophy.

Author Biography

Ana Maria Abreu-Velez, 4048764897

Dr. Abreu has more than 28 years of health science, biomedical research and translational scientific experience involving human subjects with extended experience working in the fields, jungles with communities from the bench to the clinic and clinical trials to the formulation of programs-projects, protocols translating scientific discoveries into public health action in several areas. There is a great gap between the volume of scientific and public health knowledge generated through research and the application of that research in community settings. Dr. Abreu has been able to apply that knowledge by creating local, State, National and International projects, programs, booklets instructions, guidance working with patients, communities, health providers, community leaders, Shamans, private donors, and none-profit and for profit organizations and authorities in her field of expertise. She has applied those skills on a program of a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America (Orphan diseases protected under The Orphan Drug Act law of 1983 of the United States) Also in programs to control and prevent sexual transmitted diseases, as well as in areas of high violence in inner cities cooperating with several religious group to decrease disease and violence Dr. Abreu leadership contributed to decrease the morbimortality in people affected by autoimmune blistering diseases from almost 97% of morbimortality to less than 3% working multidisciplinary. Dr. Abreu’s experienced in creating goals and objectives for a public health organization including the two larger Institutes of Tropical Diseases in Colombia at the University of Antioquia and at the Institute of Health Science, and as Director of Autoimmune and Blistering National Diseases Programs. Dr. Abreu scientific leadership is reflected in publications in peer-reviewed journals, including 100 first-authored papers (57 are now under PUBMED, 46 in review on Medline, 30 in preparation and 70 more in process of being added as a coauthor. Usually scientist at the highest international level rarely achieve more than 20 or 30 peer-reviewed journals as first authors in their entire productive scientific life. Dr. Abreu also had written a book and multiple book chapters. Dr. Abreu is often consulted in policy initiative and served as a national and/or international expert on public health science scientific research, private firms, governmental and none profit organizations. Dr. Abreu works alongside teams of people disseminating of evidence-based guidelines and effectively increasing awareness about several diseases. Dr. Abreu is considered a world leader in autoimmune skin blistering diseases and skin Immunodermatology and the leader in the study of a new variant of endemic pemphigus foliaceus (an orphan disease) the father of Immunodermatology Dr. Ernest H Beutner, PhD, whom died in recent years, gave her the torch of this field for her scientific and ethical services. This is quite an honor, coming from the person who discovered the autoimmune nature of pemphigus, pemphigoids, psoriasis, lupus band etc. For her groundbreaking achievements and break sin many dogmas in skin autoimmunity she is considered among the top in the world leaders in her research field. Dr. Abreu also discovered and characterized clinically, epidemiologically, immunological, genetically and environmentally new variant of endemic pemphigus foliaceus. She has also discovered new proteins, autoantigens, new junctions, and neural receptors/mechanoreceptors among many other accomplishments including developing of multiple complex immunological and molecular techniques. Dr. Abreu has more than 18 years of experience in management and leadership positions(s) that include administration, budget oversight, human resources management, liaison activities, and strategic leadership, publications in peer-reviewed journals, including 100 first-authored papers. Dr. Abreu has knowledge of, or experience within managed care and health care settings and, more generally, a strong understanding of the business needs of complex organizations. Dr. Abreu has more than 28 years of experience as a scientist, physician-immunologist, dermatologist and expert in clinical trials, drug regulations tropical diseases and disease prevention. Dr. Abreu has more than 18 years in executive management; advising university presidents, boards of trustees, and advocates, as well as mentoring interns, pre-graduate students, fellows, physicians, scientist, PhD, post-doctoral fellows working multidisciplinary. Dr. Abreu has very high ethical standards. Dr. Abreu has led and overseen the implementation of several institutional long and short term goals in accordance with institutional strategies and risks, and managed these risks.

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