Toxic epidermal necrolysis (TEN), also known as Lyell's syndrome , [1] is a rare, life- threatening skin condition that is usually caused by a reaction to drugs. The disease causes the top layer of skin (the epidermis) to detach from the lower layers of the skin (the dermis ), all over the body, leaving the body susceptible to severe infection. The case fatality ratio ranges from 25 to 30%, and death
usually occurs as a result of sepsis and
subsequent multiorgan system failure .
Treatment primarily involves discontinuing the use of causative agent(s), and supportive care in either the intensive care unit or burn unit of
a hospital.
Signs and symptoms
TEN ultimately results in extensive skin
involvement with redness , necrosis , and detachment of the top (epidermal) layer of the skin and mucosa. Before these severe findings develop, people often have a flu-like prodrome ,
with a cough, runny nose, fever, decreased appetite and malaise.
Cause
Drug reactions have been reported to cause 80-95% of TEN cases.
The drugs most often implicated in TEN are:
antibiotics
sulfonamides ( sulfamethoxazole ,
sulfadiazine , sulfapyridine )
beta-lactams ( cephalosporins , penicillins,
carbapenems )
nonsteroidal anti-inflammatory drugs
allopurinol
antimetabolites ( methotrexate )
antiretroviral drugs (nevirapine)
corticosteroids
anxiolytics ( chlormezanone )
anticonvulsants ( phenobarbital , phenytoin ,
carbamazepine , and valproic acid ). [2]
TEN has also been reported to result from infection with Mycoplasma pneumoniae ,dengue virus. Contrast agents used in imaging studies as well as transplantation of bone marrow or organs have also been linked to TEN development.
Treatment
The primary treatment of TEN is
discontinuation of the causative factor(s), usually an offending drug, early referral and management in burn units or intensive care units , supportive management, and nutritional
support.
Current literature does not convincingly
support use of any adjuvant systemic therapy. Initial interest in Intravenous immunoglobulin (IVIG) came from research showing that IVIG
could inhibit Fas-FasL mediated keratinocyte apoptosis in vitro. Unfortunately, research studies reveal conflicting support for use of IVIG in treatment of TEN. Ability to draw
more generalized conclusions from research to date has been limited by lack of controlled trials, and inconsistency in study design in
terms of disease severity, IVIG dose, and
timing of IVIG administration. [3] Larger, high quality trials are needed to assess the actual benefit of IVIG in TEN.
Numerous other adjuvant therapies have been tried in TEN including, corticosteroids , cyclosporin , cyclophosphamide ,
plasmapheresis, pentoxifylline , N-
acetylcysteine , ulinastatin, infliximab , and Granulocyte colony-stimulating factors (if TEN associated- leukopenia exists). There is mixed evidence for use of corticosteriods and scant evidence for the other therapies.
