Stevens-Johnson Syndrome (Causes, Risk factors, and Complications)

Stevens-Johnson Syndrome (Causes, Risk factors, and Complications)

Definition: Stevens-Johnson syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. The mucous membranes of the eyes, mouth, and/or genitals are also commonly affected. Together with toxic epidermal necrolysis (TEN) and Stevens-Johnson/toxic epidermal necrolysis (SJS/TEN), it forms a spectrum of disease, with SJS being less severe.

Stevens-Johnson syndrome is usually caused by an unpredictable adverse reaction to certain medications. It can also sometimes be caused by an infection. SJS is at the less severe end of the spectrum, and TEN is at the more severe end. It is considered SJS when skin detachment involves less than 10% of the body surface, and TEN when skin detachment involves more than 30% of the body surface. People with skin detachment involving 10-30% of the body surface are said to have “SJS/TEN overlap.” All forms of SJS/TEN are a medical emergency that can be life-threatening.

The syndrome often begins with flu-like symptoms, followed by a red or purple rash that spreads and forms blisters. The affected skin eventually dies and peels off.  Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia, and multiple organ failure.

Stevens-Johnson syndrome is a medical emergency that usually requires hospitalization. Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications as people’s skin regrows.

Recovery after Stevens-Johnson syndrome can take weeks to months, depending on the severity of your condition. If it was caused by a medication, people will need to permanently avoid that drug and others closely related to it.

Causes and Risk factors of Stevens-Johnson syndrome: Stevens-Johnson syndrome (SJS) is a rare and unpredictable reaction. It is thought to arise from a disorder of the immune system. The immune reaction can be triggered by drugs or infections. Genetic factors are associated with a predisposition to SJS. The cause of SJS is unknown in one-quarter to one-half of cases. SJS, SJS/TEN, and TEN are considered a single disease with common causes and mechanisms.

In children, Stevens-Johnson syndrome is usually triggered by a viral infection, such as:

  • mumps
  • flu
  • herpes simplex virus, which causes cold sores
  • Coxsackie virus, which causes Bornholm disease
  • Epstein-Barr virus, which causes glandular fever

Less commonly, bacterial infections can also trigger the syndrome.

Medications (Drugs) that can cause Stevens-Johnson syndrome include:

  • Anti-gout medications, such as allopurinol
  • Medications to treat seizures and mental illness (anticonvulsants and antipsychotics), with added risk if people also undergo radiation therapy;
  • Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve)
  • Medications to fight infection, such as penicillin
  • Infectious causes

Infections that can cause Stevens-Johnson syndrome include:

  • Herpes virus (herpes simplex or herpes zoster)
  • Pneumonia
  • HIV
  • Hepatitis A

It’s important to emphasize that Stevens-Johnson syndrome is rare and the overall risk of getting the syndrome is low, even for people using these medicines.

Risk factors for Stevens-Johnson syndrome may include:

  • viral infections – such as herpes, hepatitis, viral pneumonia or HIV
  • a weakened immune system – as a result of HIV or AIDS, autoimmune conditions, such as lupus, or certain treatments, such as chemotherapy and organ transplants
  • a previous history of Stevens-Johnson syndrome – if the syndrome was previously caused by medication, people are at risk of it reoccurring if they take the same medication again, or medications from the same family of medications
  • a family history of Stevens-Johnson syndrome – if a close family member has had the syndrome, people’s risk of getting it may be increased

If people have a gene called HLA-B 1502, they have an increased risk of Stevens-Johnson syndrome, particularly if they take certain drugs for seizures, gout or mental illness. Families of Chinese, Southeast Asian or Indian descent are more likely to carry this gene.

Complications of Stevens-Johnson syndrome: Stevens-Johnson syndrome (SJS) is a rare condition, with a reported incidence of around 2.6 to 6.1 cases per million people per year. In the United States, about 300 new diagnoses are made each year. The condition is more common in adults than in children.

Stevens-Johnson syndrome complications include:

  • Secondary skin infection (cellulitis).
  • Blood infection (sepsis).
  • Eye problems.
  • Lung involvement.
  • Permanent skin damage.

In some people, SJS/TEN is fatal. The overall mortality (death) rate is about 25%, ranging from about 10% for SJS to over 30% for TEN. The most common causes of death include sepsis, acute respiratory distress syndrome, and multiple organ failure.

 

Information Source:

  1. rarediseases.info.nih.gov
  2. mayoclinic.org
  3. nhs.uk
  4. Wikipedia