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一例伴有显著眼部受累和抗双链 DNA 阳性的 Parry-Romberg 综合征和 en coup de sabre 重叠病例。

An overlap case of Parry-Romberg syndrome and en coup de sabre with striking ocular involvement and anti-double-stranded DNA positivity.

机构信息

Department of Dermatology, Ministry of Health, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey.

Department of Pathology, Ministry of Health, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey.

出版信息

Indian J Ophthalmol. 2018 Feb;66(2):336-338. doi: 10.4103/ijo.IJO_196_17.

Abstract

Parry-Romberg syndrome (PRS) may overlap localized scleroderma (morphea) lesions with linear depression (en coup de sabre [ECDS]). Overlap case with PRS and ECDS was presented. Enophthalmos, uveitis, ocular torticollis, keratic linear precipitates, and anti-double-stranded DNA positivity were identified. Subendothelial keratic precipitates detected by an in vivo laser scanning confocal microscopy were the first profiled in the literature. Patients must be evaluated and followed up carefully by their clinics to prevent misdiagnosis and unnecessary procedures such as surgery of ocular torticollis as muscular torticollis.

摘要

进行性脂肪营养不良-硬皮病综合征(PRS)可能与线状凹陷(En coup de sabre [ECDS])的局限性硬皮病(硬斑病)病变重叠。本文报告了一例 PRS 和 ECDS 重叠病例。存在眼球内陷、葡萄膜炎、眼性斜颈、角膜线状沉积物和抗双链 DNA 阳性。通过活体激光扫描共聚焦显微镜检测到的角膜内皮下沉积物是文献中首次报道的。患者必须由其诊所进行仔细评估和随访,以防止误诊和不必要的程序,如眼性斜颈的手术治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d824/5819137/85fe666115fa/IJO-66-336-g001.jpg

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