RT Book, Section
A1 Kane, Kay Shou-Mei
A1 Nambudiri, Vinod E.
A1 Stratigos, Alexander J.
SR Print(0)
ID 1133126139
T1 PRIMARY BULLOUS DERMATOSES
T2 Color Atlas & Synopsis of Pediatric Dermatology, 3e
YR 2016
FD 2016
PB McGraw-Hill Education
PP New York, NY
SN 9780071843942
LK accesspediatrics.mhmedical.com/content.aspx?aid=1133126139
RD 2024/04/18
AB Epidermolysis  bullosa  (EB)  defines  a  group  of  rare  inherited  mechanobullous  skin  disorders  that  are  characterized  by  skin  fragility  and  bullae  formation.  There  are  three  major  categorizations  of  the  disease:  epidermolysis  bullosa  simplex  (EBS),  junctional  epidermolysis  bullosa  (JEB),  and  dystrophic  epidermolysis  bullosa  (DEB)  with  over  20  different  phenotypes  representing  mutations  in  the  genes  of  at  least  18  structural  proteins  of  the  skin  (in  the  epidermis,  dermal–epidermal  junction,  or  upper  papillary  dermis).