Toxic epidermal necrolysis syndrome, also known as TEN or Lyell's syndrome, is a sudden, life-threatening necrotizing skin reaction caused by certain medications or infections. TEN syndrome is a very rare disease, but it occurs after taking relatively frequently used medications. In the event of its occurrence, quick diagnosis is extremely important, because if left untreated, it quickly leads to serious organ complications and even death.

Contents:

  1. Toxic epidermal necrolysis syndrome - causes
  2. Toxic epidermal necrolysis syndrome - symptoms
  3. Toxic epidermal necrolysis syndrome - diagnosis
  4. Toxic epidermal necrolysis syndrome - treatment
  5. Toxic epidermal necrolysis syndrome - prognosis

Toxic epidermal necrolysis syndrome(akaTEN syndromeorLyell syndrome ), it's quick loss of epidermis over a large area of ​​the body, which occurs most often under the influence of medications.

There are three disease entities in diagnostics, which are differentiated on the basis of the body surface area covered by skin lesions. If necrosis is related to:

  • <10% ciała stwierdza się Zespół Stevensa-Johnsona
  • 11-29% of the body, then overlap syndrome is diagnosed
  • >30% has toxic epidermal necrolysis syndrome discussed here

TEN syndrome, apart from a larger body surface area covered by skin lesions, is also characterized by a much more severe course and increased mortality. In Stevens Johnson's syndrome, the mortality rate is around 5%, while in TEN's it is up to 40%.

Toxic epidermal necrolysis syndrome - causes

The use of certain medications and infections are proven causes of toxic epidermal necrolysis. Drugs that may contribute to TEN include:

  • antibiotics from the group of sulfonamides, fluoroquinolones, cephalosporins and penicillins.
  • anticonvulsants - phenobarbital, lamotrigine, carbamazepine, phenytoin
  • allopurinol - a drug used to treat gout
  • non-steroidal anti-inflammatory drugs - this group includes most over-the-counter pain relievers and antipyretics

For the probable cause of the toxic syndromeepidermal necrolysis is also considered to be an infection with the microorganismMycoplasma Pneumoniae .

Since this infection most often occurs in children and adolescents, it is in them that toxic necrolysis of the epidermis caused by infections occurs most often. The TEN syndrome in the course of infection is usually non-specific - the symptoms are then less severe, and the skin lesions mainly affect the mucous membranes.

Toxic epidermal necrolysis syndrome is a very rare disease - the incidence is estimated at 0.4-1.2 cases per million people a year.

The disease can occur at any age and gender, but studies have shown a slightly more common incidence in women.

HIV carriers are particularly predisposed to develop TEN - the risk of developing the disease increases a thousandfold in them.

Another group of patients with an increased risk of this syndrome are elderly people with other diseases and people with immunodeficiencies - this is most likely due to the use of multi-drug therapies.

Toxic epidermal necrolysis syndrome - symptoms

Disease can develop in two ways. The first is the presence of symptoms that resemble a minor infection, such as:

  • fever
  • sore throat
  • cough
  • muscle and joint pain

The second is a sudden onset, where the first symptom of the disease is extensive changes on the skin.

The skin changes initially take the form of erythematous changes - there is redness and swelling, initially on the face, limbs, and then on the trunk.

The next stage of the disease is the appearance of blisters at the site of the erythema, which break easily, leaving an oozing erosion.

A very characteristic symptom in the course of TEN syndrome is flaky peeling of seemingly he althy skin after rubbing it. This syndrome also has a tendency to affect the mucous membranes, this occurs 1-3 days after the appearance of skin lesions.

Initially, changes on the mucous membranes appear in the oral cavity - then they take the form of hemorrhagic scabs, which, passing to the mucosa of the gastrointestinal tract, prevent food intake.

The course of the disease also causes changes in the eyesight, leading to conjunctivitis, inflammation of the eyeball, ulceration and scarring.

There is also photophobia and excessive dryness of the eyeball.

All these symptoms, if left untreated, can lead to loss of vision.

Involvement of the urethral mucosa causes stricturesand scars that may make it difficult or even impossible to urinate.

General symptoms are related to the presence of changes in the mucous membranes - sore throat, difficulty eating or defecating, there may also be difficulties in breathing due to the development of respiratory failure.

TEN syndrome also has a high fever.

Toxic epidermal necrolysis syndrome - diagnosis

Diagnosis is based on clinical symptoms.

It is primarily the presence of extensive, creeping blisters and the flaky separation of the epidermis of a large area of ​​the body, as well as the sudden appearance of skin lesions accompanied by high fever and a general severe condition of the patient, which is most often due to dehydration.

An important factor influencing the diagnosis is the patient's medical history, which indicates that he or she is taking medications that may cause TEN syndrome.

It is assumed that the most likely causative agent is the drug started about 2-3 weeks before the appearance of the first skin lesions, however, antiepileptic drugs and allopurinol are usually tolerated during the first weeks of use before they induce toxic epidermal necrolysis.

In the case of antibiotics and non-steroidal anti-inflammatory drugs, the reaction is much faster and more violent. Since toxic epidermal necrolysis can be caused by over-the-counter medications, it is very important to inform your doctor about all medications you have recently taken.

Toxic epidermal necrolysis syndrome - treatment

Treatment depends on the severity of the symptoms and the severity of the patient's condition, if you suspect that toxic epidermal necrolysis is suspected, contact your doctor immediately, as it is crucial to implement appropriate treatment promptly.

First of all, the drug suspected of inducing toxic epidermal necrolysis should be discontinued.

It is generally accepted that the procedure should be the same as in the case of treatment of 2nd degree burns, i.e. it should be based on intensive hydration, because due to the loss of a large amount of epidermis, which protects against water evaporation, it is very quick and essential dehydration.

Body erosions should be covered with non-stick gauze, body temperature should be maintained and exposure to microorganisms avoided to prevent bacterial contamination of skin lesions.

Pain relieving and antipyretic treatment is also recommended, and if oral nutrition is not possible, apply nutrition bygastric probe.

In the case of changes in the mucous membranes of the eye, ophthalmic treatment should be instituted immediately, as these changes rapidly lead to blindness. Sometimes patients require treatment in an intensive care unit.

Toxic epidermal necrolysis syndrome - prognosis

Mortality in the TEN syndrome reaches 40%, the key is the rapid implementation of appropriate treatment and prevention of complications.

Toxic epidermal necrolysis syndrome is a disease in which the skin and internal organs are affected, which can be life-threatening.

Medicines, including those available without a prescription, are the main causative factor, so always in the event of any disturbing symptoms after taking medications, you should contact your doctor, because the most important role in the prognosis is the quick start of treatment.

Category:

Dermatology