Open Access Case report

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

Carolin Appelhans1*, Frank Breuckmann1, Andreas Bastian2, Peter Altmeyer1 and Alexander Kreuter1

Author Affiliations

1 Dept. of Dermatology, Ruhr-University Bochum, Gudrunstr. 56, D-44791 Bochum, Germany

2 Dept. of Internal Medicine, St. Josef Hospital Bochum, Gudrunstr. 56, D-44791 Bochum, Germany

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BMC Dermatology 2004, 4:17  doi:10.1186/1471-5945-4-17

Published: 8 November 2004

Abstract

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.