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

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 orga...

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Autores principales:	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
Formato:	article
Lenguaje:	EN
Publicado:	Mattioli1885 2019
Materias:	areae compositae of the heart endemic pemphigus foliaceus MYZAP left cardiac hypertrophy p0071 ARVCF Dermatology RL1-803
Acceso en línea:	https://doaj.org/article/0a487de6333a475893d1740188794e79
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spelling	oai:doaj.org-article:0a487de6333a475893d1740188794e792021-11-17T08:29:19ZInvolvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus10.5826/dpc.0903a022160-9381https://doaj.org/article/0a487de6333a475893d1740188794e792019-07-01T00:00:00Zhttp://dpcj.org/index.php/dpc/article/view/708https://doaj.org/toc/2160-9381 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. Ana Maria Abreu-VelezYulieth A. Upegui-ZapataCarlos A. Valencia-YepesEduardo Upegui-QuicenoAlejandra M. Jiménez-EchavarríaCésar D. Niño-PulidoBruce R. SmollerMichael S. HowardMattioli1885articleareae compositae of the heartendemic pemphigus foliaceusMYZAPleft cardiac hypertrophyp0071ARVCFDermatologyRL1-803ENDermatology Practical & Conceptual, Vol 9, Iss 2 (2019)
institution	DOAJ
collection	DOAJ
language	EN
topic	areae compositae of the heart endemic pemphigus foliaceus MYZAP left cardiac hypertrophy p0071 ARVCF Dermatology RL1-803
spellingShingle	areae compositae of the heart endemic pemphigus foliaceus MYZAP left cardiac hypertrophy p0071 ARVCF Dermatology RL1-803 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 Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus
description	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.
format	article
author	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
author_facet	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
author_sort	Ana Maria Abreu-Velez
title	Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus
title_short	Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus
title_full	Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus
title_fullStr	Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus
title_full_unstemmed	Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus
title_sort	involvement of the areae compositae of the heart in endemic pemphigus foliaceus
publisher	Mattioli1885
publishDate	2019
url	https://doaj.org/article/0a487de6333a475893d1740188794e79
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Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus

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