1例Ⅰ型多发性神经纤维瘤儿童的多发性肌纤维瘤和表皮疣状痣表现  

Multiple myofibromas and an epidermal verrucous nevus in a child with neurofibromatosis type 1

作　　者：De Schepper S. Janssens S. Messiaen L. J.-M. Naeyaert 党倩丽 

机构地区：[1]Department of Dermatology,De Pintelaan 185,BE-9000Ghent,Belgium Dr.

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2005年第2期16-16,共1页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Neurofibromatosis type 1 (NF1) is a common neurocutaneous autosomal dominant genetic disorder affecting primarily tissues derived from the embryonic neural crest. Two hallmark features of NF1 are the wide range of potentially affected tissues and the enormous phenotypic variability of disease traits even among patients from the same family. We present a boy fulfilling the diagnostic criteria for NF1 with two unusual lesions: infantile myofibromatosis and a verrucous epidermal nevus. To our knowledge this association has never been described before.Neurofibromatosis type 1 (NF1) is a common neurocutaneous autosomal dominant genetic disorder affecting primarily tissues derived from the embryonic neural crest. Two hallmark features of NF1 are the wide range of potentially affected tissues and the enormous phenotypic variability of disease traits even among patients from the same family. We present a boy fulfilling the diagnostic criteria for NF1 with two unusual lesions: infantile myofibromatosis and a verrucous epidermal nevus. To our knowledge this association has never been described before.

关 键 词：肌纤维瘤 疣状痣 外胚叶 疾病特征 神经性疾病 神经嵴 组织范围 标志性特征 诊断标准 婴儿期 

分 类 号：R739.4[医药卫生—肿瘤]