Toxic epidermal necrolysis
Toxic epidermal necrolysis: Excerpt from Professional Guide to Diseases (Eighth Edition)
Toxic epidermal necrolysis (TEN) is a rare, severe skin disorder that causes epidermal erythema, superficial necrosis, and skin erosions. Mortality is high (30%), especially among debilitated and elderly patients. Reepithelialization is slow, and residual scarring is common. TEN primarily affects adults. Some experts consider TEN to be a maximal form of Stevens-Johnson syndrome (SJS), with SJS being a maximal variant of erythema multiforme major.
Causes and incidence
In 80% of cases, TEN is determined to result from a drug reaction — most commonly to sulfonamides, penicillins, barbiturates, hydantoins, procainamide, isoniazid, nonsteroidal anti-inflammatory drugs, or allopurinol. Numerous other drugs have also been implicated, although 5% of patients with TEN report no drug use. It may also result from chemical exposure, viral infection, mycoplasma pneumonia, or immunization.
TEN may reflect an immune response, or it may be related to overwhelming physiologic stress (coexisting sepsis, neoplastic diseases, and drug treatment).
The annual worldwide incidence of TENS is 1 to 3 cases for every 1 million people.
Signs and symptoms
Early symptoms include inflammation of the mucous membranes, a burning sensation in the conjunctivae, malaise, fever, and generalized skin tenderness. After such prodromal symptoms, TEN erupts in three phases:
❑ diffuse, erythematous rash
❑ vesiculation and blistering
❑ large-scale epidermal necrolysis and desquamation.
Large, flaccid bullae that rupture easily expose extensive areas of denuded skin, permitting both loss of tissue fluids and electrolytes and widespread systemic involvement. Systemic complications may include bronchopneumonia, pulmonary edema, GI and esophageal hemorrhage, shock, renal failure, sepsis, and disseminated intravascular coagulation; these conditions markedly increase the likelihood of mortality.
Diagnosis
Confirming diagnosis Early diagnosis is very important and is based on the patient’s clinical status at the peak stage of the disease. Nikolsky’s sign (skin sloughs off with slight friction) is present in erythematous areas. Culture and Gram stain of lesions determine whether infection is present. Supportive findings include leukocytosis, elevated levels of alanine aminotransferase and aspartate aminotransferase, albuminuria, and fluid and electrolyte imbalances.
Exfoliative cytology and biopsy aid in ruling out erythema multiforme and exfoliative dermatitis.
Treatment
Treatment consists of transferring the patient to a burn center or an intensive care unit and providing I.V. fluid replacement to maintain fluid and electrolyte balance. Xenografts should be used to prevent pain and infection and to provide the framework for reepithelialization. High doses of I.V. immunoglobulins may halt progression if given early in the course of illness. Steroids may be appropriate initially, but should be discontinued as soon as healing occurs. Use of steroids may decrease survival rates only secondary to increased incidence of infections and other complications. Necrotic skin should be débrided. The patient also should stop using suspected drugs.
Special considerations
❑ Frequently assess hematocrit and hemoglobin, electrolyte, serum protein, and blood gas levels.
❑ Monitor vital signs, central venous pressure, and urine output. Watch for signs of renal failure (decreased urine output) and bleeding. Report fever immediately, and obtain blood cultures and sensitivity tests promptly, as ordered, to detect and treat septic infection.
❑ Prevent secondary infection with appropriate precautions. Use systemic antibiotics for specific identified infections only.
❑ Maintain skin integrity as much as possible. The patient shouldn’t wear clothing and should be covered loosely to prevent friction and sloughing of skin. A low air-loss or air-fluidized bed is helpful.
❑ Administer analgesics as needed. Wounds will be virtually pain-free after the dermis is covered by the xenograft.
❑ Provide eye care hourly to remove exudate. Because ocular lesions are common, the ophthalmologist should examine the patent’s eyes daily.
❑ Ensure that suspected drugs are never administered.
❑ Encourage the patient to wear a medical alert bracelet.
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
» Next page: Staphylococcal scalded skin syndrome (Professional Guide to Diseases (Eighth Edition))
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