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[眼睑弹性组织离解。上睑下垂的罕见原因]

[Elastolysis of the eyelids. A rare cause of ptosis].

作者信息

Emesz M, Wohlfart C, Schaeppi H, Kiesler J, Thaller-Antlanger H

机构信息

Landesklinik für Augenheilkunde und Optometrie, Salzburg.

出版信息

Ophthalmologe. 2004 May;101(5):509-13. doi: 10.1007/s00347-003-0897-9.

Abstract

BACKGROUND

Mid-dermal elastolysis is a rare disorder of elastic tissue which is characterized clinically by wrinkling of the skin and histologically by the selective absence of elastic fibers in the mid dermis. Two patients with severe ptosis and a loss of the lateral tarsal suspension are reported.

PATIENTS AND METHODS

A 36-year-old male patient presented with bilateral ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. With normal clinical and routine laboratory investigations, punch biopsy revealed clear signs of mid-dermal elastolysis. Plastic surgery of both eyelids (levator advancement) was performed with good success after a 1-year interval without progression of ptosis. Another 37 year old patient suffered from mild ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. As the patient chose not to have surgical treatment, he was treated with oral corticosteroids. To differentiate between an acute and a chronic type of elastolysis a punch biopsy is required. In addition, we want to highlight the surgical option in chronic and non-progressive cases.

CONCLUSIONS

In contrast to formerly reported localized forms of acute acquired cutis laxa with complete loss of elastic fibres in the reticular and papillary dermis, we report a chronic type of elastolysis in these patients. In our experience in cases without any apparent progression of symptoms a surgical repair can provide long-term success.

摘要

背景

真皮中层弹性组织离解是一种罕见的弹性组织疾病,临床特征为皮肤起皱,组织学特征为真皮中层弹性纤维选择性缺失。本文报告了2例患有严重上睑下垂且外侧睑板悬吊缺失的患者。

患者与方法

一名36岁男性患者出现双侧上睑下垂、眼睑皮肤萎缩及外侧睑板悬吊缺失。临床及常规实验室检查均正常,经皮穿刺活检显示有明确的真皮中层弹性组织离解迹象。1年后,对双侧眼睑进行了整形手术(提上睑肌前移术),手术成功,上睑下垂未进展。另一名37岁患者患有轻度上睑下垂、眼睑皮肤萎缩及外侧睑板悬吊缺失。由于患者选择不接受手术治疗,故对其采用口服皮质类固醇进行治疗。要区分急性和慢性弹性组织离解类型,需要进行经皮穿刺活检。此外,我们想强调在慢性和非进行性病例中的手术选择。

结论

与之前报道的局限性急性获得性皮肤松弛症不同,后者在网状真皮和乳头真皮中弹性纤维完全缺失,我们报告的这些患者为慢性弹性组织离解类型。根据我们的经验,在症状无明显进展的病例中,手术修复可取得长期成功。

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