Fibromatosis of the hand associated with EMO syndrome: A Case report
© Appelhans et al; licensee BioMed Central Ltd. 2004
Received: 06 April 2004
Accepted: 08 November 2004
Published: 08 November 2004
EMO syndrome, defined as a triad including exophthalmus, pretibial myxedema and osteoarthropathia, is a rare condition in patients suffering from hyperthyreosis.
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.
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.
EMO syndrome is a rare condition seen in patients suffering from hyperthyreosis. It is defined as a triad of exophthalmus, pretibial myxedema and osteoarthropathia occurring in less than 1% of patients suffering from Graves' disease . We here describe an unusual case of EMO syndrome associated with unilateral fibromatosis and an initial stage of generalized myxedema of the skin.
Grave's disease is known to eventually develop after radioiodine therapy  and this may be followed by a EMO syndrome. To our knowledge this is the first report about a EMO syndrome combined with coexistent palmoulnar fibromatosis. The patient was treated with lymphdrainage and physiological compressive therapy. As Farr et al.  reported the successful use of PUVA in a case of scleromyxedema, we initiated a therapeutic attempt with conventional UVA1 irradiation five times a week for one week followed by three times per week for three consecutive weeks (20 J/cm2 single dose, 280 J/cm2 cumulative dose). While the congestion of lymph clearly improved, pretibial myxedema remained without any signs of regression.
A massive recurrence after excision of thyroid dermopathy, as described in literature, could not be observed in our patient during a follow-up of 2.5 years.
Generalized fibrosing processes are thought to be based on an accumulation of glycosaminogylcans. Common explanations comprise its initiation by autoimmune processes, e.g. thyreotropin receptors on fibroblasts, or aberrant T-cell cytokine stimulation, leading to overwhelming glycosaminoglycan production  Fibrosing processes such as retroperitoneal or sellar associated with EMO syndrome or multifocal fibrosclerosis, respectively, have been previously reported [5, 6]. Nevertheless, coexistent palmar fibromatosis so far has not been described.
Therefore, we conclude that concomitant fibromatosis might appear in various localizations requiring elaborated diagnostic procedures and monitoring in all patients affected. We started low-dose UVA1 irradiation of the pretibial myxedema known to be able to degrade pathologic collagenous architecture by inducing dermal matrix-metalloproteinases as well as by decreasing abnormal cytokine liberation following T-cell apoptosis . Nevertheless, our patient discontinued UVA1 phototherapy due to non response after a cumulative dosage of 280 J/cm2 UVA1 (seven treatment sessions). However, we suggest that phototherapy might also be considered as an adjunct therapeutic alternative in persistent EMO syndrome.
- Goette DK: Thyroid acropachy. Arch Dermatol 1980, 166: 205–206. 10.1001/archderm.116.2.205View ArticleGoogle Scholar
- Niepomniszcze H, Pitoia F, Goodall C, Manavela M, Bruno OD: Development of Graves' hyperthyroidism after radioiodine treatment for a toxic nodule: is the hyperthyroidism always triggered by 1311 therapy? Thyroid 2001, 11: 991. 10.1089/105072501753211091View ArticlePubMedGoogle Scholar
- Farr PM, Ive FA: PUVA treatment of scleromyxoedema. Br J Dermatol 1984, 110: 347–350.View ArticlePubMedGoogle Scholar
- Chang TC, Wu SL, Hsiao YL, Kuo ST, Chien LF, Kuo YF, Change CC, Chang TJ: TSH and TSH receptor antibody-binding sites in fibroblasts of pretibial myxedema are related to the extracellular domain of entire TSH receptor. Clin Immunol Immunopathol 1994, 71: 113–120. 10.1006/clin.1994.1059View ArticlePubMedGoogle Scholar
- Armigliato M, Paolini R, Bianchini E, Monesi G, Zamboni S, D'Andrea E: Hashimoto's thyroiditis and Graves' disease associated with retroperitoneal fibrosis. Thyroid 2002, 12: 829–831. 10.1089/105072502760339415View ArticlePubMedGoogle Scholar
- van der Pol R, Nieuwenhuis MG, Mourits MP: Multifocal fibrosclerosis presenting as Grave's orbitopathy. Bilateral exophthalmos associated with retroperitoneal and sellar fibrosis. Graefes Arch Clin Exp Ophthalmol 1999, 237: 256–258. 10.1007/s004170050227View ArticlePubMedGoogle Scholar
- Dawe RS: Ultraviolet A1 phototherapy. Br J Dermatol 2003, 148: 626–637. 10.1046/j.1365-2133.2003.05261.xView ArticlePubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-5945/4/17/prepub
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