toxic epidermal necrolysis


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Related to toxic epidermal necrolysis: Acute Generalized Exanthematous Pustulosis

Toxic Epidermal Necrolysis

 

Definition

Toxic epidermal necrolysis is a rare condition that causes large portions of the epidermis, the skin's outermost layer, to detach from the layers of skin below. A reaction to a medication is the primary cause.

Description

Toxic epidermal necrolysis (TEN) begins with fever, cough, and other nonspecific symptoms, and is soon followed by purplish, bloody-looking lesions on the skin and mucous membranes. These early lesions, typically found on the head, neck, and upper chest, soon merge and blister. Sheets of epidermis then begin to detach from the skin layers below. In time, the entire surface of the skin may be involved, with detachment of 100% of the epidermis.

Causes and symptoms

The main cause of TEN is a severe drug reaction. Some investigators believe there may be additional infectious causes. A severe reaction in transplant patients, called graft-vs.-host disease, can also produce TEN. One study reported more than 100 different drugs as causes of TEN. The drugs most commonly implicated, however, include antibacterial sulfonamides such as sulfadiazine, antibiotics such as aminopenicillins and cephalosporins, and anticonvulsants like phenytoin. TEN is extremely rare. Researchers estimate that there are 0.2 cases per million users of aminopenicillins and 4.5 cases per million users of sulfonamides.
Exactly what leads to detachment of the epidermis remains unclear. People with TEN seem to have difficulty metabolizing the offending drug. Some researchers suggest that certain substances that should be cleared from the body instead get deposited on the outer shell of the epidermis, causing an immune response that leads the body to "reject" the skin.

Diagnosis

Diagnosis is made primarily on the appearance and spread of the skin lesions, and on a history that includes introduction of a new medication within the previous one to three weeks. A biopsy of the early lesions will confirm the diagnosis. Physicians will consider other potential diseases that cause similar symptoms before reaching a diagnosis of TEN. One is erythema multiforme, a recurrent skin disorder that produces lesions similar in appearance to TEN. However, this disorder is not caused by a drug reaction and does not lead to sheet-like shedding of the skin. Another disease, Stevens-Johnson syndrome, is a drug-induced skin disease that some experts believe is really a milder form of TEN. Staphylococcal scalded skin syndrome (SSSS) also looks like TEN, but it is caused by a staphylococcal infection. Unlike TEN, which occurs rarely in children, SSSS primarily affects infants, young children, and adults with weakened immune systems.

Treatment

There is no specific treatment for TEN. Patients are typically treated in an intensive care unit or in a burn unit and receive treatment similar to that given to patients with major burns. With the loss of skin, severe dehydration is a major risk, so health care workers will attempt to replace fluids intravenously. Nutritional supplementation from a tube routed through the nose to the stomach may also be contemplated to promote the healing of the skin. Infection is a major risk, so some physicians "paint" the open lesions with topical antiseptics. Others use skin grafts taken from cadavers or cultured skin substitutes to cover large open areas until healing can occur. 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.

Key terms

Epidermis — The outermost layer of the skin.
Erythema multiforme — A recurrent skin disorder that produces lesions similar in appearance to TEN, but is not caused by a drug reaction and does not lead to sheet-like shedding of the skin.
Staphylococcal scalded skin syndrome — A disease caused by Staphylococcus aureus, in which large sheets of skin may peel away from the body. It most often affects infants, young children, and people with weakened immune systems.
Stevens-Johnson syndrome — A drug-induced skin disease that some experts believe is really a milder form of TEN.

Prognosis

About 25-30% of patients with TEN die. Elderly patients, those with extensive skin lesions, and those with AIDS have the worst prognosis. Widespread systemic infection (sepsis) is the primary cause of death. Survivors, however, will be completely healed in three to four weeks.

Prevention

There is no prevention for TEN. No reliable test can indicate that a specific drug may cause TEN in a specific patient. Some researchers believe skin tests of potentially offending drugs may prove useful in the future.

Resources

Organizations

American Academy of Dermatology. 930 N. Meacham Road, P.O. Box 4014, Schaumburg, IL 60168-4014. (847) 330-0230.Fax: (847) 330-0050. http://www.aad.org.

necrolysis

 [nĕ-krol´ĭ-sis]
separation or exfoliation of necrotic tissue.
toxic epidermal necrolysis an exfoliative skin disease in which erythema spreads rapidly over the body, followed by blisters much like those seen in a second degree burn. It may be caused by drug reactions, infections (viral, bacterial, or fungal), neoplastic disease, graft-versus-host reaction, and chemical exposure.

tox·ic ep·i·der·mal ne·crol·y·sis (TEN),

a syndrome in which a large portion of the skin becomes intensely erythematous with epidermal necrosis and peels off in the manner of a second-degree burn, often simultaneous with the formation of flaccid bullae, resulting from drug sensitivity or of unknown cause; the level of separation is subepidermal, unlike staphylococcal scalded skin syndrome in which there is subcorneal change.
Synonym(s): Lyell syndrome

toxic epidermal necrolysis (TEN)

a rare life-threatening skin disease characterized by epidermal erythema, superficial necrosis, and skin erosions. This condition, which affects mainly adults, makes the skin appear scalded, often leaving scars. The cause of TEN is unknown, but it may result from toxic or hypersensitive reactions, an immune response, or severe physiological stress. A similar skin disorder may be the result of a staphylococcal infection. Treatment of TEN commonly involves the administration of IV fluids to replace body fluids and maintain electrolyte balance. Frequent laboratory analyses are necessary to monitor hematocrit and hemoglobin, serum proteins, electrolytes, and blood gases. Also called scalded skin syndrome. Compare staphylococcal scalded skin syndrome.
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Toxic epidermal necrolysis

toxic epidermal necrolysis

Lyell syndrome Dermatology An acute life-threatening mucocutaneous reaction often to drugs–80% of cases, characterized by widespread and/or confluent erythema, necrosis, formation of multiple bullae that coalesce, followed by mucocutaneous sloughing Etiology Drug hypersensitivity–eg, allopurinol, anticonvulsants, barbiturates, carbamazepine, NSAIDs–eg, phenylbutazone, oxicam derivatives, sulfonamides–eg, T-S, infections, vaccination, RT, CA Clinical Prodrome–fever, malaise, erythema, followed by subepidermal bullae, epidemal sloughing Complications Dehydration, electrolyte imbalance or 'third space phenomenon', abscess formation, sepsis, renal failure, CHF, GI hemorrhage, shock, skin failure, oral–erosive stomatitis, anogenital, conjunctival–keratitis, corneal erosions, respiratory tract lesions; ±30% are fatal Treatment Symptomatic, as with 2nd degree burns, stop offending drug. See Erythema multiforme, Stevens-Johnson syndrome. Cf Staphylococcus scalded skin syndrome.

tox·ic ep·i·der·mal ne·crol·y·sis

(TEN) (tok'sik ep'i-dĕr'măl nĕ-krol'i-sis)
A syndrome in which a large portion of the skin becomes intensely erythematous, with epidermal necrosis and flaccid bullae, resulting from drug sensitivity or of unknown cause.
Synonym(s): Lyell syndrome.

toxic epidermal necrolysis

A severe skin disorder featuring redness, peeling, blistering and loosening and shedding of large sheets of the EPIDERMIS. In children it was believed to be usually due to a circulating staphylococcal toxin and is often called the staphylococcal scalded skin syndrome (SSSS). It is now thought that the condition is almost always a severe reaction to a drug. The condition is also known as Lyell disease after the dermatologist Alan Lyell who first described it in 1956.

Lyell,

Alan, 20th century Scottish dermatologist.
Lyell disease - a disease affecting infants in which large areas of skin peel off as a result of upper respiratory staphylococcal infection. Synonym(s): Ritter disease; Ritter syndrome; staphylococcal scalded skin syndrome
Lyell syndrome - a syndrome in which a large portion of the skin becomes intensely erythematous with epidermal necrosis, and peels off. Synonym(s): toxic epidermal necrolysis

necrolysis

separation or exfoliation of necrotic tissue.

toxic epidermal necrolysis
an acute exfoliative disease of skin and mucous membranes in dogs, cats and monkeys. Characterized by full thickness epidermal necrosis and accompanied by erythema, vesicles, bullae and ulcers, and systemic signs of fever, anorexia and lethargy. It is associated with concurrent infections or neoplasia, and drug reactions.

toxic

poisonous; pertaining to poisoning.

toxic algae
see algal poisoning.
toxic biotransformations
enzymatic changes of nontoxic to toxic substances, usually in the liver.
toxic epidermal necrolysis
see toxic epidermal necrolysis.
toxic fat syndrome
see chicken edema disease.
toxic granulation
see toxic granules.
toxic hepatitis, toxic liver disease
caused by a very large number of poisons including inorganic, organic, plant.
toxic myopathy
uncommon but is caused by e.g. gossypol, Cassia spp., monensin and the other ionophore coccidiostats.
toxic nephrosis
caused by many toxins, e.g. mercury, arsenic, copper, aminoglycoside antibiotics.
toxic shock
see toxemic shock.
toxic shock syndrome
see toxemic shock.
References in periodicals archive ?
A clinicoetiological study of steven-johnson syndrome and toxic epidermal necrolysis.
There is still no consensus on a definite treatment method for toxic epidermal necrolysis and SJS (2).
Risk factors for the development of ocular complications of Stevens- Johnson syndrome and toxic epidermal necrolysis.
Acute Management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis to Minimize Ocular Sequelae.
StevensJohnson syndrome and toxic epidermal necrolysis in new
Another worker reported fixed drug eruptions in 28 Percent , followed by Stevens-Johnson syndrome/ toxic epidermal necrolysis in 18.
Teraki Y, Murota H, Izaki S: Toxic epidermal necrolysis due to zonisamide associated with reactivation of human herpesvirus 6.
An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children.
Within days, she began exhibiting signs of toxic epidermal necrolysis, a skin disease that burned off 90% of her skin and left her blind.
To illustrate, one of the patients identi-fied by Dr Foster to be in this category was a patient admitted into one of UHB's specialist services with a condition called toxic epidermal necrolysis, which is known to have a mortality in excess of 50 per cent.