Steven-Johnson Syndrome

Mar 31, 2022

Table of contents

Mucosa is a layer of soft tissue found in all parts of the digestive system from the mouth to the anus, in the genital tract and in the eyes. Stevens Johnson syndrome is a disease that affects the mucous membranes, skin and genital organs. Although callus is seen, it follows a serious clinical picture.

Steven Johnson syndrome is often caused by side effects of certain medications. In addition, some infections can also cause the disease to occur.

The first symptoms of the disease are similar to those of influenza. However, if it progresses, it causes blisters, red and purple rashes that spread more and more. Eventually, the skin tissues affected by these lesions die and fall off.

Steven Johnson syndrome is a medical emergency and is treated in hospital burn units or intensive care units. The purpose of treatment varies depending on the underlying cause.

Erythema multiforme is a disease caused by chest infections and the herpes virus and has similar features to Steven Johnson syndrome. However, it presents a milder clinical picture.

Symptoms of Steven Johnson Syndrome

The most characteristic symptom of Steven Johnson syndrome is skin pain. The symptoms of this stage of the disease, which starts with flu-like symptoms, are as follows.

  • Cough,
  • Joint pain,
  • Headache
  • Fever of 38 degrees,
  • Not feeling well in general,

A few days after these symptoms appear, other symptoms caused by the disease begin to appear. Symptoms in the advanced stage are as follows.

  • Individually formed spots with a darker center and lighter periphery,
  • Itching is not observed,
  • The spots multiply rapidly, within a few days or hours,
  • In the next stage, large bubbles begin to form,
  • After the blisters burst, they leave painful sores,
  • Facial swelling
  • Swelling of the lips and the formation of crusted sores,
  • The mucous membranes in the mouth, throat and genital area can also develop blisters and ulcers,
  • Dehydration
  • Affection of the eyes,
  • Corneal ulcer,

The causes of Steven Johnson syndrome in children are usually the following:

  • Epstein-Barr virus causing glandular fever,
  • Coxsackie virus causing Bornholm disease,
  • The herpes virus that causes cold sores,
  • A cold,
  • Mumps
  • Rarely, some bacterial infections,

Steven Johnson syndrome in adults is usually seen as a side effect of medication. The most commonly used medicines that cause this side effect are the following:

  • Sulfasalazine,
  • Sertraline,
  • Sulfamethoxazole and other sulfa antibiotics,
  • Phenytoin,
  • Phenobarbital,
  • Oxycam,
  • Nevirapine
  • Lamotrigine,
  • Carbamazepine
  • Allopurinol

Steven Johnson Risk Factors

Some factors increase the risk of the rare Steven Johnson syndrome. These risk factors are as follows:

  • Viral infections,
  • Weakened immune system (conditions such as HIV, AIDS, autoimmune diseases, chemotherapy, organ transplantation)
  • A history of Steven Johnson syndrome,
  • Family history of Steven Johnson syndrome,
  • Genes have been identified that increase the risk of Steven Johnson syndrome in some people.

How is Steven Johnson Syndrome Diagnosed?

Steven Johnson syndrome is diagnosed by dermatologists. A physical examination, symptoms and medical history are usually combined to make a diagnosis. To confirm the diagnosis, a biopsy sample can be taken from the skin.

Treatment of Steven Johnson Syndrome

Steven Johnson syndrome needs to be treated as soon as possible. Otherwise, symptoms progress rapidly and become life-threatening. If the disease is severe, patients are treated in burn or intensive care units. In addition, patients must first stop taking their medications. In some cases, however, it is not possible to determine which medication caused Steven Johnson syndrome. In these cases, patients should stop all medications. During treatment, symptom-oriented therapies are applied.

Painkillers

Steven Johnson syndrome causes severe skin pain. Strong painkillers are used to alleviate this.

Cold and moist compresses applied to the skin

The dead layer formed after the blisters is gently removed and a sterile dressing is then applied.

Moisturizers

Cold moisturizers are constantly applied to the skin.

Liquid Loading

In order to prevent dehydration and ensure nutrition, patients are provided with nutrients through a feeding tube.

Gargle

Antiseptic or anesthetic mouthwashes are used to temporarily numb the inside of the mouth and allow patients to swallow.

Corticosteroids

To control skin inflammation, corticosteroid therapy is administered as a course of tablets or topically.

Antibiotics

It is included in the treatment if sepsis is suspected.

Eye Drops

When the cause of Steven Johnson syndrome is found and stopped, the skin reaction will stop. The formation of new skin tissues starts to grow within a few days. However, Steven Johnson syndrome can take weeks or even months to fully recover. The duration of recovery is determined by the severity of the disease. Patients may also feel tired for a few weeks after discharge.

Complications of Steven Johnson Syndrome

Steven Johnson syndrome causes the skin and mucous membranes to be severely affected, which can lead to complications. These complications are as follows:

  • The skin may change color as the skin at the site of the wounds regrows. Although it is rare, some patients may have scars.
  • It can cause more serious infections such as cellulitis and sepsis.
  • It can cause inflammation of internal organs and diseases such as hepatitis and pneumonia.
  • It can lead to corneal ulcers or blindness.
  • Narrowing of the vaginal area may occur due to the accumulation of scar tissue.
  • Scars may remain on the penis.

How to Prevent Steven Johnson Syndrome?

If Steven Johnson syndrome is caused by a reaction to a medication, then that medication or group of medications should be avoided for the rest of the patient’s life.

If there is a genetic predisposition in the family, it is recommended that other families avoid using the same group of drugs.

To avoid a recurrence of Steven Johnson syndrome, the symptoms need to be carefully monitored.

People of Southeast Asian, Chinese and Indian descent should have genetic testing before taking medicines such as carbamazepine and allopurinol.

Ask the Doctor a Question