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Fibromatosis of the hand associated with EMO syndrome: A Case report

BACKGROUND: EMO syndrome, defined as a triad including exophthalmus, pretibial myxedema and osteoarthropathia, is a rare condition in patients suffering from hyperthyreosis. CASE PRESENTATION: We here describe an interesting case of EMO syndrome associated with unilateral fibromatosis of the hand an...

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Autores principales: Appelhans, Carolin, Breuckmann, Frank, Bastian, Andreas, Altmeyer, Peter, Kreuter, Alexander
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2004
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC529452/
https://www.ncbi.nlm.nih.gov/pubmed/15533248
http://dx.doi.org/10.1186/1471-5945-4-17
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author Appelhans, Carolin
Breuckmann, Frank
Bastian, Andreas
Altmeyer, Peter
Kreuter, Alexander
author_facet Appelhans, Carolin
Breuckmann, Frank
Bastian, Andreas
Altmeyer, Peter
Kreuter, Alexander
author_sort Appelhans, Carolin
collection PubMed
description BACKGROUND: EMO syndrome, defined as a triad including exophthalmus, pretibial myxedema and osteoarthropathia, is a rare condition in patients suffering from hyperthyreosis. CASE PRESENTATION: We here describe an interesting case of EMO syndrome associated with unilateral fibromatosis of the hand and an initial stage of generalized myxedema of the skin. To our knowledge a similar case has not yet been described in literature though reports about associated fibromatosis, e.g. located retroperitoneally, already exist. Familiar explanations include its initiation by autoimmune processes or aberrant T-cell cytokine stimulation leading to an overwhelming production of glycosaminoglycans. CONCLUSION: Interpreting our case in context with previous reports we conclude that associated fibromatosis induced by autoimmune processes may affect a variety of different localizations and therefore requires careful monitoring. A therapeutical attempt by using UVA1 irridation for pretibial myxedema remained without a satisfying regression.
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spelling pubmed-5294522004-11-21 Fibromatosis of the hand associated with EMO syndrome: A Case report Appelhans, Carolin Breuckmann, Frank Bastian, Andreas Altmeyer, Peter Kreuter, Alexander BMC Dermatol Case Report BACKGROUND: EMO syndrome, defined as a triad including exophthalmus, pretibial myxedema and osteoarthropathia, is a rare condition in patients suffering from hyperthyreosis. CASE PRESENTATION: We here describe an interesting case of EMO syndrome associated with unilateral fibromatosis of the hand and an initial stage of generalized myxedema of the skin. To our knowledge a similar case has not yet been described in literature though reports about associated fibromatosis, e.g. located retroperitoneally, already exist. Familiar explanations include its initiation by autoimmune processes or aberrant T-cell cytokine stimulation leading to an overwhelming production of glycosaminoglycans. CONCLUSION: Interpreting our case in context with previous reports we conclude that associated fibromatosis induced by autoimmune processes may affect a variety of different localizations and therefore requires careful monitoring. A therapeutical attempt by using UVA1 irridation for pretibial myxedema remained without a satisfying regression. BioMed Central 2004-11-08 /pmc/articles/PMC529452/ /pubmed/15533248 http://dx.doi.org/10.1186/1471-5945-4-17 Text en Copyright © 2004 Appelhans et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Appelhans, Carolin
Breuckmann, Frank
Bastian, Andreas
Altmeyer, Peter
Kreuter, Alexander
Fibromatosis of the hand associated with EMO syndrome: A Case report
title Fibromatosis of the hand associated with EMO syndrome: A Case report
title_full Fibromatosis of the hand associated with EMO syndrome: A Case report
title_fullStr Fibromatosis of the hand associated with EMO syndrome: A Case report
title_full_unstemmed Fibromatosis of the hand associated with EMO syndrome: A Case report
title_short Fibromatosis of the hand associated with EMO syndrome: A Case report
title_sort fibromatosis of the hand associated with emo syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC529452/
https://www.ncbi.nlm.nih.gov/pubmed/15533248
http://dx.doi.org/10.1186/1471-5945-4-17
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