Two Cases of Segmental Neurofibromatosis.
- Author:
Chang Geun CHO
;
Il Hwan KIM
;
Hae Jun SONG
;
Chil Hwan OH
- Publication Type:Case Report
- Keywords:
Segmental neurofibromatosis
- MeSH:
Adult;
Cafe-au-Lait Spots;
Female;
Humans;
Middle Aged;
Neurofibroma;
Neurofibromatoses*;
Population Characteristics
- From:Korean Journal of Dermatology
1998;36(6):1083-1087
- CountryRepublic of Korea
- Language:Korean
-
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.
