Qureshi Farhan, Dharmasena Aruna, Leatherbarrow Brian
Department of Ophthalmology, Manchester Royal Eye Hospital, Manchester, United Kingdom.
Ophthalmic Plast Reconstr Surg. 2015 Sep-Oct;31(5):e119-20. doi: 10.1097/IOP.0000000000000144.
Scleromyxedema is characterized by cutaneous mucinosis and rarely presents to oculoplastic surgeons with bilateral upper and lower eyelid swelling. The authors present 2 case reports with a review of the literature and discuss the ophthalmic manifestations of scleromyxedema and the management of these cases. Both patients were in their early 50s and were fit and well prior to the presentation. They both presented with eyelid swelling and underlying nodularity of the subcutaneous tissue. The skin biopsy confirmed cutaneous manifestations of scleromyxedema. The systemic manifestations responded to intravenous and oral steroids and intravenous infusions of immunoglobulin. The ophthalmic manifestations were managed conservatively. Scleromyxedema is a rare connective tissue disease characterized by cutaneous mucinosis, extracutaneous manifestations, and monoclonal gammopathy. It rarely affects the eyelids and cornea. The authors would like to present 2 cases of periocular scleromyxedema.
硬化性黏液水肿的特征为皮肤黏液变性,很少有双侧上、下眼睑肿胀的情况出现在眼整形医生面前。作者呈现2例病例报告并进行文献综述,讨论硬化性黏液水肿的眼部表现及这些病例的处理。两名患者均为50岁出头,就诊前身体健康。他们均表现为眼睑肿胀及皮下组织的潜在结节。皮肤活检证实为硬化性黏液水肿的皮肤表现。全身表现对静脉及口服类固醇以及静脉输注免疫球蛋白有反应。眼部表现采用保守治疗。硬化性黏液水肿是一种罕见的结缔组织疾病,其特征为皮肤黏液变性、皮肤外表现及单克隆丙种球蛋白病。它很少累及眼睑和角膜。作者特此呈现2例眼周硬化性黏液水肿病例。