伴有视网膜血管炎和黄斑水肿的硬化性黏液水肿

SCLEROMYXEDEMA WITH RETINAL VASCULITIS AND MACULAR EDEMA.

作者信息

Gutiérrez-Aguirre Karen I, Stewart Michael W, Sluzevich Jason C

机构信息

*Department of Medicine and Nutrition, University of Guanajuato, Leon Guanajuato, Mexico; and Departments of †Ophthalmology, and ‡Dermatology, Mayo Clinic, Jacksonville, Florida.

出版信息

Retin Cases Brief Rep. 2017;11(4):319-322. doi: 10.1097/ICB.0000000000000353.

DOI:10.1097/ICB.0000000000000353

PMID:27348792

Abstract

BACKGROUND/PURPOSE: To describe the findings and clinical course of a patient with scleromyxedema complicated by retinal vasculitis and macular edema.

METHODS

Interventional case report.

RESULTS

A 64-year old Caucasian woman with recently diagnosed, biopsy proven scleromyxedema presented with decreased visual acuity (20/50 OD; 20/100 OS) due to retinal vasculitis and macular edema. She received intravitreal bevacizumab and subtenon's triamcinolone acetonide for the macular edema, and bilateral pars plana vitrectomies were required for vitreous hemorrhages. Monthly intravenous infusions of immunoglobulins (IVIG) resulted in resolution of the macular edema and vasculitis, and stabilization of the VA (20/40 OD; counting fingers at 6 feet OS).

CONCLUSION

Scleromyxedema may be complicated by retinal vasculitis and macular edema. Treatment with corticosteroids and vascular endothelial growth factor inhibitors may be minimally effective but IVIG should be considered for both the ocular and systemic findings.

摘要

背景/目的：描述一名合并视网膜血管炎和黄斑水肿的硬化性黏液水肿患者的检查结果及临床病程。

方法

介入性病例报告。

结果

一名64岁的白种女性，近期诊断为经活检证实的硬化性黏液水肿，因视网膜血管炎和黄斑水肿出现视力下降（右眼20/50；左眼20/100）。她接受了玻璃体内注射贝伐单抗和球周注射曲安奈德治疗黄斑水肿，因玻璃体出血需要行双侧扁平部玻璃体切除术。每月静脉输注免疫球蛋白（IVIG）使黄斑水肿和血管炎消退，视力稳定（右眼20/40；左眼在6英尺处数指）。