Exophthalmus-Myxoedema Circumscriptum Praetibiale-Osteoarthropathia Hypertrophicans (E. M. O.) Syndrome in Graves' Disease: A Review of Eight Cases Reported in Japan

Saito, Shintaro; Sato, Toshiro; Yamamoto, Makiko; Yamaguchi, Tohru; Yoshida, Katsumi; Sasai, Yohichiro; Yoshinaga, Kaoru

doi:10.1620/tjem.115.155

Cited by 6 publications

(2 citation statements)

References 12 publications

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“…Exophthalmos, myxedema, and osteoarthropathy (EMO) syndrome is a triad that was first described by Thomas in 1933 [ 3 ]. This entity is a very rare condition that affects less than 1% of patients with thyroid disease [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Utility of rituximab treatment for exophthalmos, myxedema, and osteoarthropathy syndrome resistant to corticosteroids due to Graves’ disease: a case report

Ferreira‐Hermosillo¹,

Casados-V²,

Paúl-Gaytán³

et al. 2018

J Med Case Reports

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BackgroundExophthalmos, myxedema, and osteoarthropathy syndrome is a very rare condition that is associated with Graves’ disease. The presence of dermopathy and the involvement of joint/bone tissues indicate that it seems to be related with the severity of the autoimmune process. Owing to its low incidence, there is a lack of information regarding its treatment and clinical follow-up. Some cases improved after use of high doses of steroids; however, some patients do not respond to this treatment. Recently, the effectiveness of rituximab for treatment of Graves’ ophthalmopathy resistant to corticosteroids has been demonstrated. However, it has never been used for the treatment of exophthalmos, myxedema, and osteoarthropathy syndrome (particularly for the treatment of osteoarticular manifestations).Case presentationWe present the case of a 54-year-old Mexican woman previously treated for Graves’ disease who developed post-iodine hypothyroidism and exophthalmos, myxedema, and osteoarthropathy that did not improve after high doses of steroids (intravenous and oral). Her exophthalmos, myxedema, and osteoarthropathy syndrome symptoms improved as early as 6 months after treatment with rituximab.ConclusionExophthalmos, myxedema, and osteoarthropathy syndrome is a non-classical presentation of Graves’ disease, whose clinical manifestations could improve after treatment with rituximab, particularly in those patients with lack of response to high doses of corticosteroids.

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Section: Introductionmentioning

confidence: 99%

Utility of rituximab treatment for exophthalmos, myxedema, and osteoarthropathy syndrome resistant to corticosteroids due to Graves’ disease: a case report

Ferreira‐Hermosillo¹,

Casados-V²,

Paúl-Gaytán³

et al. 2018

J Med Case Reports

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“…When myxedema occurs as part of a triad of extrathyroidal manifestations that also includes exophthalmos and osteoarthropathy, it constitutes EMO syndrome, which is observed in <1% of patients with autoimmune thyroid disease (5). The extrathyroidal symptoms of EMO tend to develop chronologically with an onset of eye disease followed by myxedema and then finally osteoarthropathy or acropachy in cases of a long duration (6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

Radiation therapy as part of the therapeutic regimen for extensive multilocular myxedema in a patient with exophthalmos, myxedema and osteoarthropathy syndrome: A case report

et al. 2015

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Abstract. Exophthalmos, myxedema and osteoarthropathy (EMO) comprise the triad known as EMO syndrome, which is rarely observed in patients with autoimmune thyroid disease. The present study reports the case of a patient with EMO, including the response of this rare combination to radiotherapy. A 48-year-old patient with EMO syndrome presented to the Department of Radiation Oncology, University Hospital of Muenster, eight years prior to writing with therapy-resistant pretibial myxedema and hypertrophic osteoarthropathy of the metacarpal bones. The patient had been diagnosed with Graves' disease (GD) 26 years prior to presentation, which was treated by thyroidectomy and radioiodine therapy. Four years subsequent to the diagnosis of GD, the patient developed exophthalmos, which was treated using radiotherapy. An evident pretibial, foot and hand myxedema developed within the 10 years following the onset of orbitopathy. The skin lesions were treated using radiation therapy subsequent to the failure of multiple surgical procedures and medical treatments. Almost eight years subsequent to the administration of irradiation, no recurrence was observed on the lower right leg, nor was any recurrence on the lower left leg observed approximately four years subsequent to the completion of radiotherapy. However, an additional lesion on the left hand demonstrated slow progression following treatment with radiation therapy. The present study indicates that radiation therapy can be considered as adjuvant therapy for patients with refractory myxedema, to prevent or delay the recurrence of myxedema subsequent to surgical excision.

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Triad of exophthalmos, pretibial myxedema, and acropachy in a patient with Graves' disease

Anderson

Miller

2003

Journal of the American Academy of Dermatology

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Exophthalmus-Myxoedema Circumscriptum Praetibiale-Osteoarthropathia Hypertrophicans (E. M. O.) Syndrome in Graves' Disease: A Review of Eight Cases Reported in Japan

Cited by 6 publications

References 12 publications

Utility of rituximab treatment for exophthalmos, myxedema, and osteoarthropathy syndrome resistant to corticosteroids due to Graves’ disease: a case report

Utility of rituximab treatment for exophthalmos, myxedema, and osteoarthropathy syndrome resistant to corticosteroids due to Graves’ disease: a case report

Radiation therapy as part of the therapeutic regimen for extensive multilocular myxedema in a patient with exophthalmos, myxedema and osteoarthropathy syndrome: A case report

Triad of exophthalmos, pretibial myxedema, and acropachy in a patient with Graves' disease

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