RT Book, Section
A1 Mockenhaupt, Maja
A1 Roujeau, Jean-Claude
A2 Kang, Sewon
A2 Amagai, Masayuki
A2 Bruckner, Anna L.
A2 Enk, Alexander H.
A2 Margolis, David J.
A2 McMichael, Amy J.
A2 Orringer, Jeffrey S.
SR Print(0)
ID 1162814448
T1 Epidermal Necrolysis (Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis)
T2 Fitzpatrick's Dermatology, 9e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9780071837798
LK dermatology.mhmedical.com/content.aspx?aid=1162814448
RD 2024/04/23
AB AT-A-GLANCEEpidermal  necrolysis  is  a  rare  life-threatening  reaction  mainly  induced  by  medication.Widespread  apoptosis  of  keratinocytes  is  provoked  by  the  activation  of  a  cell-mediated  cytotoxic  reaction  and  amplified  by  cytokines,  mainly  granulysin.Confluent  purpuric  and  erythematous  macules  evolving  to  flaccid  blisters  and  epidermal  detachment  often  start  on  the  upper  trunk  and  spread  to  the  limbs  associated  with  mucous  membrane  involvement.Histopathology  shows  full-thickness  necrosis  of  epidermis  associated  with  mild  mononuclear  cell  infiltrate.A  dozen  “high-risk”  drugs  account  for  half  of  cases.More  than  20%  of  cases  remain  idiopathic  or  may  be  caused  by  infection.Early  identification  and  withdrawal  of  suspect  medication  in  drug-induced  cases  are  essential  for  good  patient  outcomes.Treatment  consists  mainly  of  supportive  care,  but  recent  evidence  suggests  a  significant  benefit  of  immunomodulating  treatment  with  cyclosporine.The  death  rate  is  high  and  increases  with  disease  severity  and  age  of  the  patient.  The  majority  of  survivors  have  long-lasting  sequelae,  needing  systematic  follow-up  examinations.