RT Book, Section
A1 Wataya-Kaneda, Mari
A2 Kelly, A. Paul
A2 Taylor, Susan C.
A2 Lim, Henry W.
A2 Serrano, Ana Maria Anido
SR Print(0)
ID 1161549048
T1 Tuberous Sclerosis Complex
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 accessmedicine.mhmedical.com/content.aspx?aid=1161549048
RD 2024/04/20
AB KEY  POINTSTuberous  sclerosis  complex  (TSC)  is  an  autosomal  dominant  disease  characterized  by  generalized  hamartoma  formation  in  nearly  every  organ,  with  various  manifestations  occurring  throughout  the  individual’s  lifetime.Skin  manifestations  of  TSC  include  angiofibromas,  forehead  plaques,  hypomelanotic  macules,  shagreen  patches,  and  ungual  fibromas.Hypomelanotic  macules  are  observed  in  more  than  90%  of  patients  with  TSC  and  are  evident  at  birth  or  during  early  infancy.  More  than  three  hypomelanotic  macules  are  useful  for  the  diagnosis  of  TSC  in  infant.Hypomelanotic  macules  are  off-white  in  color  and  usually  oval,  polygonal,  or  ash  leaf  shaped,  but  sometimes  appear  as  scattered  numerous  small  white  macules.  Hypomelanotic  macules  present  on  the  scalp,  eyelashes,  and  eyebrows  cause  poliosis.The  hypomelanotic  macules  in  TSC  have  a  normal  number  of  melanocytes  but  decreased  number  and  size  of  melanosomes.