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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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Detalles Bibliográficos
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
Descripción
Sumario: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.