Gambichler T, Küster W, Wolter M, Rapp S, Altmeyer P, Hoffmann K
Klinik für Dermatologie und Allergologie, Ruhr-Universität Bochum, Gudrunstrasse 56, 44791 Bochum.
Hautarzt. 2000 Nov;51(11):862-4. doi: 10.1007/s001050051231.
Neurofibromatosis is a neuroectodermal systemic disease. A rare variant of this condition is bilateral segmental neurofibromatosis. A 29-year-old man presented with bilateral papillomatous plaques in the lumbar dermatomes. Clinically, the lesions were very similar to an epidermal nevus but histologic examination revealed superficial neurofibromas. Family history, ophthalmologic and neurologic investigations were unremarkable. The unusual morphologic presentation of bilateral segmental neurofibromas in this case points to the wide clinical spectrum of the disease and the significance of histologic examination in systematic nevoid lesions.
神经纤维瘤病是一种神经外胚层系统性疾病。这种疾病的一种罕见变体是双侧节段性神经纤维瘤病。一名29岁男性在腰部皮节出现双侧乳头状斑块。临床上，这些病变与表皮痣非常相似，但组织学检查显示为浅表神经纤维瘤。家族史、眼科和神经科检查均无异常。该病例中双侧节段性神经纤维瘤不寻常的形态学表现表明了该疾病广泛的临床谱以及组织学检查在系统性痣样病变中的重要性。
