RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164067442
T1 Danbolt-Closs Syndrome
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=1164067442
RD 2024/04/18
AB Inherited  vesiculobullous  disorder  characterized  by  intermittent  simultaneous  occurrence  of  diarrhea  and  bullous  periorificial  and  acral  dermatitis  (dry  lesions  surrounding  the  mouth,  ears,  nose,  and  eyes)  and  affecting  the  fingers,  feet,  and  knees.  The  skin  lesions  are  often  infected  by  Staphylococcus  aureus  and/or  Candida  albicans.  Failure  to  thrive  in  premature  babies  has  been  reported.  In  children,  there  are  periorificial  lesions  of  the  face  and  the  anogenital  region.  Alopecia  and  absence  of  eyebrows,  eyelashes,  and  the  thymus  gland  are  commonly  seen.  Affected  individuals  with  absence  of  thymus  will  present  important  anesthesia  considerations,  particularly  myasthenia  gravis  like  symptoms.