TY  - CHAP
M1  - Book, Section
TI  - Chapter 116. Basal Cell Nevus Syndrome
A1  - Oro, Anthony E.
A1  - Tang, Jean Y.
A2  - Goldsmith, Lowell A.
A2  - Katz, Stephen I.
A2  - Gilchrest, Barbara A.
A2  - Paller, Amy S.
A2  - Leffell, David J.
A2  - Wolff, Klaus
PY  - 2012
T2  - Fitzpatrick's Dermatology in General Medicine, 8e
AB  - |PrintBasal  Cell  Nevus  Syndrome  at  a  GlanceA  rare  autosomal  dominant  disorder  with  phenotypic  abnormalities  that  include  developmental  anomalies  and  postnatal  tumors,  especially  basal  cell  carcinomas  (BCCs).The  three  most  characteristic  abnormalities  are  tumors  such  as  medulloblastomas  or  BCCs,  pits  of  the  palms  and  soles,  and  odontogenic  cysts  of  the  jaw.Related  findings  include  calcification  of  the  falx  cerebri,  enlarged  body  habitus,  and  skeletal  abnormalities  of  the  ribs  and  skull.Diagnosis  is  suspected  in  a  patient  with  multiple  BCCs  arising  at  an  unexpectedly  early  age  with  an  average  age  of  onset  of  21  years.Histologic  features  of  tumors  in  BCNS  patients  are  similar  to  those  of  sporadic  BCCs  or  medulloblastomas.Several  new  therapeutic  agents,  including  SMO  antagonists  and  celecoxib,  have  been  shown  to  have  efficacy  in  treating  disease  along  with  photodynamic  therapy,  imiquimod,  and  5-fluorouracil.  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/19
UR  - accessmedicine.mhmedical.com/content.aspx?aid=56059656
ER  -