Toxic Epidermal Necrolysis

Toxic epidermal necrolysis is a life-threatening skin disorder characterized by a blistering and peeling of the top layer of skin. This disorder can be caused by a drug reaction -- most often penicillin -- or another disease.

Toxic epidermal necrolysis (TEN) is a rare, life-threatening syndrome characterized by erythema and exfoliation of the skin. TEN is usually the result of a drug hypersensitivity reaction, although it may rarely be seen as a manifestation of graftversus-host disease in a transplant recipient.

Causes

TEN is most often caused by an adverse reaction to a drug, but it sometimes arises in patients who are not taking any medications. Non-drug causes include bacterial or viral infection, malignant tumours, vaccinations or no apparent cause (idiopathic). Drugs that are known to cause TEN include:

• Antibiotics
  • Sulphonamides
  • Penicillins
  • Macrolides
  • Quinolones
• Allopurinol
• Nonsteroidal anti-inflammatory drugs (NSAIDs)
• Anticonvulsants (antiepileptic medicines)

Symptoms

• 24- to 48-hour prodrome of high fever, headache, intense malaise, myalgias, arthralgias, diarrhea, vomiting, conjunctival irritation, and skin tenderness

Signs

• Lesions are usually a morbilliform ("sandpaper") eruption or diffuse macular erythema. The skin is often tender. The lesions can progress to form vesicles, which then coalesce and slough, leaving large denuded areas.
• Mucous membranes are often involved, with erosions and sloughing occurring as well.
• Fingernails and toenails may slough or develop Beau's lines.

Treatment

The progression of the disease happens fast, usually within three days. Treatment usually includes hospitalization, often in the burn unit. If a medication is causing the skin reaction, the patient is immediately taken off the medication. With the loss of skin, severe dehydration is a major risk, so health care workers will attempt to replace fluids intravenously.

Some investigators believe system corticosteroids are useful in the treatment of TEN. But since these medications have also been implicated as a cause in some cases of TEN and are known to supress the immune system, their use should be considered carefully.