Exophthalmos, myxedema, acropachy (EMA) syndrome is a rare extrathyroidal complication of Graves' disease, affecting approximately 1% of patients.
The female-to-male ratio is 3.4:1, making this male case report exceptionally rare.
Observation:
A 52-year-old man presented with severe ophthalmopathy, pretibial myxedema, and acropachy with joint pain.
These symptoms manifested after treatments for ophthalmopathy, including thyroidectomy, retrobulbar steroid therapy, and radiotherapy.
The patient had normal thyroid function with T4 therapy, and EMA syndrome onset coincided with worsening ophthalmopathy.
Findings:
This case confirms the typical chronological sequence of extrathyroidal manifestations in autoimmune thyroid disease: thyroid disease, followed by ophthalmopathy, dermopathy, and acropachy.
Thyroid acropachy differs from other osteoarthropathies due to associated dermopathy and ophthalmopathy (EMA) and distinct subperiosteal proliferation.
Steroidal therapy effectively improved ophthalmopathy, pretibial myxedema, and acropachy, with faster resolution of exophthalmos and myxedema compared to acropachy.
Implications:
Acropachy represents the latest manifestation of EMA syndrome and correlates with ophthalmopathy exacerbation.
Traditional steroidal therapy remains an effective treatment for the multifaceted symptoms of EMA syndrome.
Understanding the chronological progression and treatment response in EMA syndrome aids in managing complex autoimmune thyroid complications.