TY - JOUR
T1 - Natural course and characteristics of cutaneous neurofibromas in neurofibromatosis 1
AU - Ehara, Yuko
AU - Yamamoto, Osamu
AU - Kosaki, Kenjiro
AU - Yoshida, Yuichi
N1 - Funding Information:
ACKNOWLEDGMENTS: This work was partly supported by a Health Labor Sciences Research Grant (Y. Y.) from the Ministry of Health, Labor and Welfare, the Platform Project for supporting in Drug Discovery and Life Science Research from the Japan Agency for Medical Research and Development (Y. Y. and K. K.) and Tottori University Faculty of Medicine Alumni Association (Y. E.).
Funding Information:
This work was partly supported by a Health Labor Sciences Research Grant (Y. Y.) from the Ministry of Health, Labor and Welfare, the Platform Project for supporting in Drug Discovery and Life Science Research from the Japan Agency for Medical Research and Development (Y. Y. and K. K.) and Tottori University Faculty of Medicine Alumni Association (Y. E.).
Publisher Copyright:
© 2017 Japanese Dermatological Association
PY - 2018/1
Y1 - 2018/1
N2 - Neurofibromatosis 1 (NF1) is characterized by cutaneous, neurological and osseous manifestations. Most NF1 patients develop cutaneous neurofibromas. However, time-dependent change with aging and the predilection site of cutaneous neurofibromas remain unclear. To clarify the natural course and characteristics of cutaneous neurofibromas, a retrospective study was conducted for 57 NF1 patients who were treated at the Department of Dermatology of Tottori University Hospital between January 2007 and April 2016. For each patient, we investigated the time-dependent changes and the numbers of cutaneous neurofibromas in four body surface regions. There was a positive correlation between age and number of cutaneous neurofibromas (r = 0.75, P < 0.001). Cutaneous neurofibromas were located on the trunk (60.2%), lower limbs (16.1%), upper limbs (14.4%), and head and neck (9.2%). There was no significant relationship between each body type (e.g. obese or thin) and cutaneous neurofibromas. With respect to the year-to-year percentage change in cutaneous neurofibromas, the average annual rate of increase was 0.21 (range, −0.71 to 1.2). The number of cutaneous neurofibromas had increased in approximately 61% of the patients 1 year later. Our data will enable physicians to estimate the overall state of cutaneous neurofibromas in NF1 and will be useful for handling cutaneous manifestations before they become a serious condition.
AB - Neurofibromatosis 1 (NF1) is characterized by cutaneous, neurological and osseous manifestations. Most NF1 patients develop cutaneous neurofibromas. However, time-dependent change with aging and the predilection site of cutaneous neurofibromas remain unclear. To clarify the natural course and characteristics of cutaneous neurofibromas, a retrospective study was conducted for 57 NF1 patients who were treated at the Department of Dermatology of Tottori University Hospital between January 2007 and April 2016. For each patient, we investigated the time-dependent changes and the numbers of cutaneous neurofibromas in four body surface regions. There was a positive correlation between age and number of cutaneous neurofibromas (r = 0.75, P < 0.001). Cutaneous neurofibromas were located on the trunk (60.2%), lower limbs (16.1%), upper limbs (14.4%), and head and neck (9.2%). There was no significant relationship between each body type (e.g. obese or thin) and cutaneous neurofibromas. With respect to the year-to-year percentage change in cutaneous neurofibromas, the average annual rate of increase was 0.21 (range, −0.71 to 1.2). The number of cutaneous neurofibromas had increased in approximately 61% of the patients 1 year later. Our data will enable physicians to estimate the overall state of cutaneous neurofibromas in NF1 and will be useful for handling cutaneous manifestations before they become a serious condition.
KW - NF1
KW - cutaneous neurofibroma
KW - distribution
KW - neurofibromatosis 1
KW - predilection body site
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U2 - 10.1111/1346-8138.14025
DO - 10.1111/1346-8138.14025
M3 - Article
C2 - 28891076
AN - SCOPUS:85028968487
VL - 45
SP - 53
EP - 57
JO - Journal of Dermatology
JF - Journal of Dermatology
SN - 0385-2407
IS - 1
ER -