File
Authors
Ehara, Yuko Division of Dermatology, Department of Medicine of Sensory and Motor Organs, School of Medicine, Tottori University Faculty of Medicine
Koga, Monji Department of Dermatology, Fukuoka University School of Medicine,
Imafuku, Shinichi Department of Dermatology, Fukuoka University School of Medicine,
Yamamoto, Osamu Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University Researchers DB KAKEN
Yoshida, Yuichi Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University Researchers DB KAKEN
Keywords
distribution
neurofibromatosis 1
plexiform neurofibroma
predilection body site
selumetinib
Abstract
Neurofibromatosis 1 (NF1) is a genetic disease characterized by cutaneous, neurological and osseous abnormalities. Approximately 20% of patients develop plexiform neurofibroma (PN), resulting in impaired quality of life. To evaluate distribution of diffuse PN on the body surface, a retrospective study was conducted for 354 patients with NF1 from 2007 to 2018 in Japan. We investigated a total of 40 patients with clinically apparent superficial diffuse PN. In the cases evaluated, 57.4% of the diffuse PN were located on the trunk, 19.2% the head and neck, 12.8% the lower limbs and 10.6% the upper limbs. Remarkably, 75.0% of the diffuse PN were located on the dorsal side. The frequency was significantly higher on the trunk than on the head and neck (P = 0.026). Our findings provide useful information for giving attention to the high possibility of diffuse PN on the dorsal side before progression in childhood and for future treatment in NF1.
Publisher
WILEY
Content Type
Journal Article
Link
ISSN
03852407
EISSN
13468138
NCID
AA00696658
Journal Title
JOURNAL OF DERMATOLOGY
Current Journal Title
JOURNAL OF DERMATOLOGY
Volume
47
Issue
2
Start Page
190
End Page
192
Published Date
2020-02
Publisher-DOI
Text Version
Author
Rights
(C) 2019 Japanese Dermatological Association
Citation
Ehara Yuko, Koga Monji, Imafuku Shinichi, et al. Distribution of diffuse plexiform neurofibroma on the body surface in patients with neurofibromatosis 1. JOURNAL OF DERMATOLOGY. 2020. 47(2). 190-192. doi:10.1111/1346-8138.15194
Department
Faculty of Medicine/Graduate School of Medical Sciences/University Hospital
Language
English
Web of Science Key ut
WOS:000502963700001