Senel Engin, Güleç Ayşe Tülin
Baskent University Faculty of Medicine, Department of Dermatology, 5. sokak No: 48 Bahcelievler, Ankara, Turkey.
Acta Dermatovenerol Alp Pannonica Adriat. 2009 Mar;18(1):21-3.
EMO syndrome is a rare extrathyroid syndrome, seen in only 1% of patients affected by extrathyroid complications of Graves' disease. A 73-year-old woman presented with a 1-year history of asymptomatic local swellings on her legs and feet. Physical examination revealed moderate proptosis and multiple, firm subcutaneous nodules of 1 to 5 cm in diameter located on the anterior and medial aspects of the shins and on feet. The patient had a history of bilateral knee arthroplasty 5 years ago. Histopathologic examination showed deposition of mucin and perivascular lymphocytic infiltration in the dermis. Dermatologic and pathologic findings were consistent with pretibial myxedema. Laboratory tests showed normal thyroid stimulating hormone (TSH) and serum free T3 and T4 levels. The TSH receptor antibody titer was elevated. Thus, with all these findings she was diagnosed with exophthalmia, myxedema, and hypertrophic osteoarthropathy (EMO) syndrome. The lesions were completely treated with three monthly intralesional corticosteroid injections and at the 4-month follow-up no recurrence was observed. Only three euthyroid cases with pretibial myxedema have been reported in the literature. Patients that have asymptomatic pretibial nodular or plaque lesions should be investigated with an ophthalmologic examination and laboratory workup to clarify a possible underlying thyroid gland disease and EMO syndrome.
EMO综合征是一种罕见的甲状腺外综合征,仅见于1%的格雷夫斯病甲状腺外并发症患者。一名73岁女性,腿部和脚部出现无症状局部肿胀1年。体格检查发现中度突眼,双侧胫骨前内侧和足部有多个直径1至5厘米的坚实皮下结节。患者5年前有双侧膝关节置换病史。组织病理学检查显示真皮内有粘蛋白沉积和血管周围淋巴细胞浸润。皮肤和病理表现符合胫前黏液性水肿。实验室检查显示促甲状腺激素(TSH)、血清游离T3和T4水平正常。TSH受体抗体滴度升高。因此,综合所有这些发现,她被诊断为突眼、黏液性水肿和肥大性骨关节病(EMO)综合征。通过每月3次病灶内注射皮质类固醇对病变进行了彻底治疗,4个月随访时未见复发。文献中仅报道了3例甲状腺功能正常的胫前黏液性水肿病例。对于有无症状的胫前结节或斑块病变患者,应进行眼科检查和实验室检查,以明确可能的潜在甲状腺疾病和EMO综合征。