RT Book, Section
A1 Yoshida, Yuichi
A2 Kelly, A. Paul
A2 Taylor, Susan C.
A2 Lim, Henry W.
A2 Serrano, Ana Maria Anido
SR Print(0)
ID 1161548982
T1 Neurofibromatosis
T2 Taylor and Kelly's Dermatology for Skin of Color, 2e
YR 2016
FD 2016
PB McGraw-Hill Education
PP New York, NY
SN 9780071805520
LK dermatology.mhmedical.com/content.aspx?aid=1161548982
RD 2024/04/19
AB KEY  POINTSNeurofibromatosis  type  1  (NF1)  is  a  neurocutaneous  genetic  disorder  caused  by  mutation  of  the  NF1  gene  on  chromosome  17q11.2,  whereas  neurofibromatosis  type  2  (NF2)  is  a  genetically  distinct  disorder  caused  by  mutation  of  the  gene  on  chromosome  22q12.2  encoding  merlin.The  worldwide  prevalence  of  NF1  is  approximately  1  in  3000  individuals  with  no  relation  to  race.NF1  is  characterized  by  café-au-lait  spots,  freckling,  neurofibromas,  Lisch  nodules,  cerebral  tumors,  and  osseous  abnormalities.Clinical  manifestations  of  NF1  are  variable  in  each  individual,  and  the  overall  degree  of  severity  and  complications  are  not  predictable.Patients  with  NF1  have  increased  risk  of  malignancies.Age-specific  annual  monitoring  and  treatment  of  complications  by  appropriate  specialists  are  important  for  management  of  NF1.