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综合征性皮脂腺痣:当前的研究发现。

Syndromic sebaceous nevus: current findings.

机构信息

Department of Pediatric Surgery, CURCP, University Hospital Center of the Canton of Vaud (CHUV), Lausanne, Switzerland.

Service of Pediatric Orthopedic Plastic Surgery, Hôpital Lapeyronie, Montpellier, France.

出版信息

Int J Dermatol. 2018 May;57(5):599-604. doi: 10.1111/ijd.13942. Epub 2018 Feb 16.

DOI:10.1111/ijd.13942
PMID:29453776
Abstract

BACKGROUND

Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar.

OBJECTIVES

The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan.

METHODS

This is a retrospective study reviewing the cases of syndromic sebaceous nevus treated in the Department of Orthopedic Plastic Pediatric Surgery in Montpellier, France, and the Department of Pediatric Surgery in Lausanne, Switzerland, between 1994 and 2016.

RESULTS

The files of six patients with syndromic sebaceous nevus were analyzed. The average age at the first consultation was 4 months. The location was craniofacial in all cases. Cerebral radiological imaging was performed on all patients; two showed abnormal findings. Four patients underwent ophthalmic examination, which all revealed abnormalities. Three patients had other associated malformations. Three patients presented with epilepsy or learning difficulties in the course of follow-up.

CONCLUSION

All patients presenting with extensive sebaceous nevus of the craniofacial region should benefit from cerebral imagery and ophthalmic examination since there is a very high probability of associated abnormalities. The developmental problems encountered could not be definitively associated with the skin malformations.

摘要

背景

皮脂腺痣是一种先天性皮肤畸形,通常发生在头皮或面部。综合征形式则很少存在与大脑和眼部畸形相关联的情况。出生时皮肤病变呈苍白色,青春期后变得不规则。在成年患者中,皮脂腺痣会发展为肿瘤(通常为良性)。在儿童时期进行手术切除可以获得更好的最终疤痕效果。

目的

本研究旨在分析我们的综合征性皮脂腺错构瘤病例,回顾现有文献,并提出治疗计划指南。

方法

这是一项回顾性研究,分析了法国蒙彼利埃矫形小儿外科系和瑞士洛桑小儿外科系 1994 年至 2016 年间治疗的综合征性皮脂腺痣病例。

结果

分析了 6 例综合征性皮脂腺痣患者的病例。首次就诊时的平均年龄为 4 个月。所有病例均位于头面部。所有患者均进行了脑部影像学检查,其中 2 例发现异常。4 例患者接受了眼科检查,均发现异常。3 例患者有其他相关畸形。3 例患者在随访过程中出现癫痫或学习困难。

结论

所有患有广泛头面部皮脂腺痣的患者都应受益于脑部影像学和眼科检查,因为存在高度关联异常的可能性。遇到的发育问题不能明确与皮肤畸形相关。

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Syndromic sebaceous nevus: current findings.综合征性皮脂腺痣:当前的研究发现。
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