Published on Nov 23, 2022 and last reviewed on Jun 13, 2023 - 4 min read
Abstract
Toxic epidermal necrolysis is a disorder that poses a potential threat to life by involving various mucous membranes.
When Stevens-Johnson syndrome (SJS) presents with severe manifestations, it is referred to as toxic epidermal necrolysis (TEN). It is a rare, life-threatening reaction of the skin. It is diagnosed when there are large areas of blistering and peeling of skin on at least 30 % of the body, causing extensive damage to the mucous membranes (the moist linings) of the eyes, mouth, and genitals. The widespread skin damage can lead to excessive loss of body fluids and may predispose to infections. It affects people of all age groups. Toxic epidermal necrolysis usually requires hospitalization, and recovery may take weeks to months. As the skin heals, supportive care is required for the patients, and this includes controlling pain, dressing the wounds, and electrolyte replacement for the patient. If the etiology of the disease was due to a medication, the particular drug must be avoided permanently.
Toxic Epidermal Necrolysis Versus Steven Johnson Syndrome
Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are similar in clinical presentation but vary in the extent of distribution. The dermal changes affect > 30 % of the body surface area in TEN and < 10 % of the body surface area in SJS; involvement of 10 % to 30 % of the body surface area is considered as an overlap of SJS and TEN.
Medications are the main etiology for over 50 % of SJS cases and up to 95 % of TEN cases. The most common drug causes include,
The sulfa group of drugs (e.g., Cotrimoxazole, Sulfasalazine).
Other antibiotics (e.g., Aminopenicillins, Fluoroquinolones, Cephalosporins).
Non-steroidal anti-inflammatory drugs (e.g., Piroxicam, Meloxicam).
Antiretroviral drugs (e.g., Nevirapine).
Antiseizure drugs (e.g., Phenytoin, Phenobarbital, Carbamazepine, Lamotrigine, Valproate).
Other miscellaneous drugs (e.g., Allopurinol, Chlormezanone).
Non-drug-related cases include,
Infection (mostly with Mycoplasma pneumoniae).
Graft-vs-host disease.
Vaccination.
In certain cases, the cause may not be identified.
Patients develop a prodrome of cough, fever, headache, malaise, and keratoconjunctivitis within the initial one to three weeks after administration of the drug that predisposes them to TEN. The first abnormality to be noted usually is the diffuse erythema of the skin. Macules suddenly appear in a target configuration, usually on the face, neck, upper trunk, and vagina. These macules eventually coalesce into large flaccid bullae and slough over a period of one to three days. Loss of epithelium is noted, including those of the nails and eyebrows, palms, and soles. Pain involving the skin, mucosa, and eye are common.
A family history of Stevens-Johnson syndrome and toxic epidermal necrolysis. A person is more susceptible to the condition if a parent or a sibling has had it.
If a drug causes this condition, there is a risk of a recurrence if used again.
Genetic Factors: Having certain genetic variations puts you at increased risk of the disease.
Patients With a Weak Immune System: The immune system can be affected by autoimmune diseases, HIV or AIDS, and organ transplants. Among people with HIV, the incidence of Stevens-Johnson syndrome and toxic epidermal necrolysis is about 100 times greater in HIV patients when compared to HIV-free individuals.
Cancer: People with cancer, especially cancers of the blood, are at increased risk of Stevens-Johnson syndrome and toxic epidermal necrolysis.
In severe cases, patients exhibit the Nikolsky sign, where layers of epithelium peel off the body at pressure points, exposing reddish and painful skin. Painful oral erosions, keratoconjunctivitis, and genital problems are seen, along with skin sloughing in about 90 % of cases. When the bronchial epithelium shows sloughing, there is a cough, dyspnea, pneumonia, pulmonary edema, and hypoxemia. Glomerulonephritis and hepatitis may develop as well.
Treatment of the condition works best when the diagnosis is made early. It is treated in an inpatient dermatologic or intensive care unit setting if it is mild to moderate and in a burn unit if it is severe.
Potentially causative drugs should be stopped immediately.
As the patients are highly susceptible to infections, they are isolated to minimize exposure and are given fluids, electrolytes, blood products, and nutritional supplements as required.
Skincare includes daily wound care similar to severe burns and prompt treatment of secondary bacterial infections.
Cyclosporine has been shown to decrease the duration of active disease by two days to three days in some instances and possibly decrease mortality.
Plasmapheresis removes reactive drug metabolites or antibodies and can be considered.
The inflammation can be reduced by the use of TNF-alpha inhibitors– Infliximab and Etanercept.
Severe cases are similar to extensive burns. It can be considered as an acute illness where the patient may be unable to eat or open the eyes and suffer massive fluid and electrolyte losses. They are more prone to infection, multiorgan failure, and death. If there is an early treatment intervention, survival rates are almost 90 %. There is a severity-of-illness score for toxic epidermal necrolysis, which systematically scores seven independent risk factors within the first 24 hours of hospitalization to determine the mortality rate for a patient and is presented as follows:
* More risk factors indicate a higher score and a higher mortality rate (%) as follows:
0–1 = 3.2 % (CI: 0.1 to 16.7)
2 = 12.1 % (CI: 5.4 to 22.5)
3 = 35.3 % (CI: 19.8 to 53.5)
4 = 58.3 % (CI: 36.6 to 77.9)
≥ 5 = > 90 % (CI: 55.5 to 99.8)
CI = confidence interval.
Conclusion:
Be aware of medicines that cause Stevens-Johnson syndrome and toxic epidermal necrolysis in the first place and avoid them to prevent another episode of TEN. Informing future healthcare providers about the history of Toxic Epidermal Necrolysis and carrying a medic alert bracelet with information about the condition will help avoid administering the drug by mistake. Recurrence of the condition could be worse and life-threatening.
Toxic epidermal necrolysis is an uncommon, life-threatening disease that mostly affects the moist lining of the mucous membranes of the skin of the eyes, mouth, and genitals. It presents with large blisters and peeling of the skin.
There are many drugs that can result in adverse reactions, like toxic epidermal necrolysis. However, antibiotics that are responsible for this skin manifestation are:
- Fluoroquinolones.
- Cephalosporin group of drugs.
- Aminopenicillins.
The confirmatory diagnosis of toxic epidermal necrolysis can be made by histopathologic analysis of the skin. Skin biopsy helps in ruling out toxic epidermal necrolysis and helps in planning the treatment accordingly.
Early recovery from toxic epidermal necrolysis can be done by early identification of the underlying cause of the disease. If any drugs causing disease are identified, they should be stopped immediately. Fluid electrolyte imbalance should be corrected by administering enough vitamins and nutritional supplements.
According to several types of research, Paracetamol is associated with an increased risk of toxic epidermal necrolysis. Hypersensitivity reactions have been observed in persons consuming Paracetamol which may also result in life-threatening conditions.
Different steps in the management of toxic epidermal Necrolysis are:
- Avoid the potential drug causing the problem.
- Correction of fluid electrolyte imbalance by administering plenty of fluid and nutritional supplements.
- Daily routine care of the affected area of the skin.
- Take the medications as prescribed by the doctor for a reduction in swelling and inflammation.
- Regular follow-up with the doctor.
The major reason for death in the case of toxic epidermal necrolysis is the sudden failure of multiple organs and sepsis. Severe sloughing of the skin epithelium can result in infections of the eyes, genitals, and mouth, resulting in respiratory failure, ocular abnormalities, and genital lesions.
The presence of large blisters and skin peeling can result in a painful and itching sensation on the skin in case of toxic epidermal necrolysis. More than itching, the blisters are painful and form on various internal or external surfaces of the mucous membranes of the skin.
Toxic epidermal necrolysis is considered a life-threatening disease as it can cause the sloughing of all skin of the mucous membranes of the eyes, genitalia, and mouth. It can result in genital lesions and eye abnormalities. It may result in death due to multiple organ failure and sepsis.
No, according to the research, there is no current relation established between the COVID virus and toxic epidermal necrolysis. Most cases of toxic epidermal necrolysis are due to triggers from drugs.
Toxic epidermal necrolysis starts within one to four weeks after one starts taking a new drug. The most common drugs that act as triggers for the disease are Fluoroquinolones, Aminopenicillins, and Cephalosporins.
No, toxic epidermal necrolysis is not contagious (do not spread from one person to another). But it causes symptoms like blisters, itching, and inflammation of the skin.
The fluids needed in toxic epidermal necrolysis are:
- Electrolyte Solution: Electrolyte solution of 7 ml per kg of body weight per percent of the area affected is administered to the patients.
- Albumin Solution: An albumin solution of around 5 ml per kg of body weight per percentage of the area affected is administered to the patients.
Last reviewed at:
13 Jun 2023 - 4 min read
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