A Case of Segmental Neurofibromatosis.
10.5021/ad.1999.11.2.109
- Author:
Gwang Seong CHOI
;
Jeung LEE
;
Duck Hwan WON
;
Sang Wahn KOO
;
Joo Heung LEE
;
Young Keun KIM
- Publication Type:Case Report
- Keywords:
Cafe-au-lait spot;
Fanconi's syndrome;
Segmental neurofibromatosis
- MeSH:
Adult;
Cafe-au-Lait Spots;
Female;
Humans;
Korea;
Neurofibroma;
Neurofibromatoses*;
Neurofibromatosis 1
- From:Annals of Dermatology
1999;11(2):109-111
- CountryRepublic of Korea
- Language:English
-
Abstract:
Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait spots and neurofibrobmas, or only neurofibroma, limited to one region of the body. Disease-associated systemic involvement is uncommon. Most patients with segmental neurofibromatosis do not have a family history of neurofibromatosis. In Korea, there have been 3 reported cases of segmental neurofibromatosis. None of them had caf6-au-fait spots, systemic involvement, or family history. This report describes a case of segmental neurofibromatosis in a 25-year-old woman who had Becker's nevus-like cafe-au-lait spots and this was linked to the presence of Fanconi's syndrome in a second degree relative of the patient.
