所讨论的综合征。

Syndrome in question.

作者信息

Rosmaninho Aristóteles, Carvalho Sandrina, Oujo Elena, Horta Miguel

机构信息

Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal.

Centro Hospitalar do Porto, Porto, Portugal.

出版信息

An Bras Dermatol. 2016 Apr;91(2):245-7. doi: 10.1590/abd1806-4841.20164231.

DOI:10.1590/abd1806-4841.20164231

PMID:27192531

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4861579/

Abstract

Neurofibromatosis is extremely variable in its presentation. Segmental neurofibromatosis (SNF), which corresponds to NF-type 5 in the Riccardi classification, is a rare disorder. It may go unrecognized if few lesions are observed. We present a case of segmental neurofibromatosis in a 28 year-old patient who presented with multiple papules and nodules distributed in dermatomal fashion on the trunk. The histopathological examination of the lesions revealed a non-encapsulated, well-circumscribed spindle cell neoplasm, which was consistent with neurofibromas.

摘要

神经纤维瘤病的临床表现极具多样性。节段性神经纤维瘤病（SNF），相当于里卡尔迪分类中的NF-5型，是一种罕见的疾病。如果观察到的病变较少，可能会被漏诊。我们报告一例28岁节段性神经纤维瘤病患者，其躯干上有多个以皮节方式分布的丘疹和结节。病变的组织病理学检查显示为非包膜性、边界清楚的梭形细胞瘤，符合神经纤维瘤。