Rosmaninho Aristóteles, Carvalho Sandrina, Oujo Elena, Horta Miguel
Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal.
Centro Hospitalar do Porto, Porto, Portugal.
An Bras Dermatol. 2016 Apr;91(2):245-7. doi: 10.1590/abd1806-4841.20164231.
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.
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。
