RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164074439
T1 Juvenile Hyaline Fibromatosis
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accesspediatrics.mhmedical.com/content.aspx?aid=1164074439
RD 2024/04/16
AB Rare  congenital  genetic  disorder  characterized  by  popular  and  nodular  skin  lesions,  soft  tissue  masses,  gingival  hypertrophy,  and  joint  contractures  of  the  large  articulations  and  bone  involvement  of  variable  complexity.  The  skin  lesions  are  slow-growing  soft  masses,  pearly  white  or  skin-colored  dermal  papules  or  subcutaneous  nodules  mostly  located  on  the  face,  scalp,  and  back.  It  is  often  confused  for  neurofibromatosis.  It  occurs  from  early  childhood  to  adulthood.  The  disease  has  a  relentlessly  progressive  course,  with  most  patients  surviving  only  up  to  the  fourth  decade.