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Toxic Epidermal Necrolysis - Cause, Symptoms, Risk Factors, Diagnosis, and Treatment

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Toxic epidermal necrolysis is a disorder that poses a potential threat to life by involving various mucous membranes.

Written by

Dr. Geethika. B

Medically reviewed by

Dr. Kaushal Bhavsar

Published At November 23, 2022
Reviewed AtDecember 27, 2023

What Is Toxic Epidermal Necrolysis?

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 percent 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, including controlling pain, dressing wounds, and electrolyte replacement. 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 more than 30 percent of the body surface area in TEN and < 10 percent of the body surface area in SJS; involvement of 10 to 30 percent of the body surface area is considered an overlap of SJS and TEN.

What Causes Toxic Epidermal Necrolysis?

Medications are the main etiology for over 50 percent of SJS cases and up to 95 percent 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.

What Are the Symptoms of Toxic Epidermal Necrolysis?

  • Prodrome Development: Patients experience a prodrome characterized by cough, fever, headache, malaise, and keratoconjunctivitis within the initial one to three weeks after drug administration, increasing the risk of Toxic Epidermal Necrolysis (TEN).

  • Initial Skin Abnormality: The first noticeable abnormality is diffuse erythema of the skin, serving as an early indicator of potential TEN development.

  • Target Configuration Macules: Macules suddenly manifest in a target configuration, typically appearing on the face, neck, upper trunk, and vagina.

  • Coalescence into Bullae: Over time, these macules coalesce into large, flaccid bullae, creating a significant clinical feature.

  • Epithelial Sloughing: Extensive sloughing of epithelial tissues occurs, involving not only the skin but also the nails, eyebrows, palms, and soles, contributing to the severity of the condition.

  • Painful Manifestations: Patients commonly experience pain affecting the skin, mucosa, and eyes, adding to the overall discomfort associated with the condition.

What Are the Risk Factors for Toxic Epidermal Necrolysis?

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: Certain genetic variations increase the risk of the disease.

  • Patients With a Weak Immune System: The immune system can be affected by autoimmune diseases, Human Immunodeficiency Virus (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.

What Are the Manifestations in Severe Cases of 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 percent 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.

What Are the Treatment and Management for Toxic Epidermal Necrolysis?

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.

  • Patients highly susceptible to infections 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 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 using TNF-alpha inhibitors– Infliximab and Etanercept.

Management of Toxic Epidermal Necrolysis includes the following:

The management of Toxic Epidermal Necrolysis (TEN) includes:

  • Immediate Hospitalization: Swift admission to a specialized unit for close monitoring.

  • Discontinuation of Causative Medications: Stop the medications causing TEN to prevent symptom escalation.

  • Supportive Care: Provide a sterile environment, proper wound care, and prevent dehydration.

  • Nutritional Support: Offer parenteral or enteral nutrition to meet metabolic needs.

  • Pain Management: Prioritize effective pain relief.

  • Psychosocial Support: Offer assistance to the patient, including counseling and additional support as necessary.

  • Ophthalmologic Consultation: Address specific symptoms and prevent ocular complications.

  • Long-Term Follow-Up: Ensure ongoing monitoring for potential ocular and psychological complications.

What Is the Prognosis for Toxic Epidermal Necrolysis?

Severe cases are similar to extensive burns. It can be considered 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 percent. The severity of Toxic Epidermal Necrolysis (TEN) is assessed through a scoring system, indicating mortality rates based on risk factors within the first 24 hours:

  • 0–1 Score: 3.2% Mortality Rate (CI: 0.1 to 16.7)

  • 2 Score: 12.1% Mortality Rate (CI: 5.4 to 22.5)

  • 3 Score: 35.3% Mortality Rate (CI: 19.8 to 53.5)

  • 4 Score: 58.3% Mortality Rate (CI: 36.6 to 77.9)

  • ≥ 5 Score: >90% Mortality Rate (CI: 55.5 to 99.8)

CI - Confidence Interval: A confidence interval is a statistical way of expressing that the range within the calculated value (an average or percentage) is likely to fall.

What Are The Long-Term Effects of Toxic Epidermal Necrolysis?

Toxic Epidermal Necrolysis (TEN) can have enduring effects, including:

  • Skin Scarring: Long-lasting skin scarring is common due to damage during the acute phase, impacting both appearance and function.

  • Ocular Complications: Involvement of the eyes can result in chronic dry eyes, light sensitivity (photophobia), and vision issues.

  • Psychological Impact: The trauma of TEN can lead to persistent psychological effects, including anxiety, Post-Traumatic Stress Disorder (PTSD), and depression.

  • Medication Sensitivities: Individuals may develop sensitivities to certain medications and need to avoid specific drugs to prevent recurrence.

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 medical 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.

Frequently Asked Questions

1.

Where Is Toxic Epidermal Necrolysis Found?

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.

2.

Which Antibiotic Can Result in Toxic Epidermal Necrolysis?

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.

3.

How Can One Diagnose Toxic Epidermal Necrolysis?

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.

4.

How to Recover From Toxic Epidermal Necrolysis?

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.

5.

Can Paracetamol Lead to Toxic Epidermal Necrolysis?

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.

6.

How Is Epidermal Necrolysis Managed?

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.

7.

What Is the Major Cause of Death in Ten?

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.

8.

Does Toxic Epidermal Necrolysis Cause Itching?

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.

9.

Why Is Toxic Epidermal Necrolysis a Life-Threatening Disease?

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.

10.

Can COVID Lead To Toxic Epidermal Necrolysis?

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.

11.

How Does Toxic Epidermal Necrolysis Get Initiated?

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.

12.

Can Toxic Epidermal Necrolysis Spread?

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.

13.

What Are the Fluids Needed in Toxic Epidermal Necrolysis?

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.
Source Article IclonSourcesSource Article Arrow
Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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