Toxic epidermal necrolysis

Toxic epidermal necrolysis
Toxic epidermal necrolysis
Classification and external resources
Specialty	dermatology
ICD-10	L51.2
ICD-9-CM	695.15
OMIM	608579
DiseasesDB	4450
eMedicine	emerg/599 med/2291 derm/405
Patient UK	Toxic epidermal necrolysis
MeSH	D004816
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Toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, 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.

The incidence of TEN is between 0.4 and 1.9 cases per million each year. TEN exists on a continuum with Stevens–Johnson syndrome (SJS). The condition is called TEN when >30% of the body surface area is involved. It is called SJS when <10% of body surface area is involved. The intermediate form, with 10 to 30 percent body surface area involvement, is called "SJS/TEN." Although there was initially debate about whether TEN and SJS fall on a spectrum of disease that includes erythema multiforme (EM), they are now considered separate conditions.

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. A history of drug exposure exists on average 14 days (ranging from 1–4 weeks) prior to the onset of symptoms, but may result as early as 48 hours if it is a reexposure.

Initial skin findings include red-purple, dusky, flat spots known as macules that start on the trunk and spread out from there. These skin lesions then transform into large blisters. The affected skin can then become necrotic or sag from the body and peel off in great swaths.

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