Two Cases of Segmental Neurofibromatosis / 대한피부과학회지

ABSTRACT

Neurofibromatosis(NF) is a disorder characterized by its relative commonness, variability, and heterogeneity. It is usually expressed as a generalized form, but is rarely localized in a limited part of the body as a segmental form. In 1981, Riccardi classified NF into 8 types and a segmental form (type V) was defined by limitation of cafe-au-lait spots and/or neurofibroma on a given unilateral segment of the body. Recently we came across two cases of typical segmental neurofibromatosis. The First case was a 53-year-old woman with localized neurofibromatosis on the right T8,9 dermatome. The Second case was a 34-year-old man with localized neurofibromatosis on the right T5,6 dermatome. There were no cafe-au-lait spots, axillary frecklings, Lisch nodules or a family history in both cases. We report these rare cases with a literature review.