Toxic epidermal necrolysis

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Toxic Epidermal Necrolysis (TEN) is a life-threatening, and usually drug-induced, dermatological condition. It is characterized by the detachment of the top layer of skin, (epidermis) from the lowers layer of the skin (dermis) all over the body. It lies at the most severe end of a spectrum of disorders that have a similar manifestation, with Stevens-Johnson syndrome and erythema multiforme.

Pathogenesis

Microscopically, TEN causes cell death throughout the epidermis. Keratinocytes, which are the cells found lower in the dermis, specialize in holding the skin cells together, undergo extensive cell death.

Etiology

Toxic epidermal necrolysis is a very rare and usually severe adverse reaction to certain drugs. The drugs most often implicated in TEN are certain antibiotics (sulfonamides, trimethoprim-sulfamethoxazole, quinolones), acetaminophen, certain seizure drugs (carbamazepine, phenytoin, valproic acid), and corticosteroids. Can also cause TEN

Symptoms

TEN affects many parts of the body, but it most severely affects the mucous membranes, such as the mouth, eyes, and vagina. The severe findings of TEN are often preceded by 1 to 2 weeks of fever. These symptoms may mimic those of a common upper respiratory tract infection. When the rash appears it may be over large and varied parts of the body, and it is usually warm and appears red. In hours, the skin becomes painful and the epidermis can be easily peeled away from the underlying dermis. The mouth becomes blistered and eroded, making eating difficult and sometimes necessitating feeding through a nasogastric tube through the nose or a gastric tube directly into the stomach. The eyes are affected, becoming swollen, crusted, and ulcerated.

Diagnosis

Clinical, Histopathology

Treatment

First Line: early withdrawal of culprit drugs, early referral and management in burn units or intensive care units,supportive management, nutritional support

Second Line: Intravenous immunoglobulin (IVIG) - Uncontrolled trials showed promising effect of IVIG on treatment of TEN; Randomized control trial is needed in the future to determine the efficacy of IVIG in TEN.

Third Line: Cyclosporin, Cyclophosphamide, Plasmapheresis, Pentoxifylline, N-acetylcysteine, Ulinastatin, Infliximab, Granulocyte colony-stimulating factors (if TEN associated-leucopenia)

(Systemic steroids are unlikely to offer any benefits)

References

• Cohen, Victor and Schwartz, Robert A. Toxic Epidermal Necrolysis eMedicine

See also

• Stevens-Johnson syndrome

External links

• Merck Home 18-203e
• Stevens Johnson Syndrome Foundation
• Stevens-Johnson syndrome Support
• DermNetNZ

survivor

• http://www.suntimes.co.za/2004/01/18/news/news13.asp