What are the other Names for this Condition? (Also known as/Synonyms)

• SJS (Steven-Johnson Syndrome)

What is Stevens-Johnson Syndrome? (Definition/Background Information)

• Steven-Johnson Syndrome (SJS) is a life-threatening skin condition caused by an adverse reaction to medication or an infection. The condition can affect individuals of all ages, however it is more prevalent in children and young adults
• The risk factors for developing Steven-Johnson Syndrome include certain viral infections and one’s genetic make-up. A host of medications can cause the condition
• Steven-Johnson Syndrome can result in severe symptoms that include red and blistered skin around the lips and mouth, as well as swelling of the face. A full physical examination and a skin biopsy are needed to diagnose an individual with Steven-Johnson Syndrome
• The treatment for Steven-Johnson Syndrome includes discontinuation of the causative medications, fluid replacement, and extended periods of hospitalization
• With rapid and adequate treatment, the prognosis of Steven-Johnson Syndrome is excellent; the survival rate is about 90%

Who gets Stevens-Johnson Syndrome? (Age and Sex Distribution)

• Steven-Johnson Syndrome has an incidence rate of 1-2 per million people per year
• It is more prevalent in children and young adults
• More cases of Steven-Johnson Syndrome have been reported in females than males
• The disorder affects all races and ethnicities and no preference is seen

What are the Risk Factors for Stevens-Johnson Syndrome? (Predisposing Factors)

The risk factors for developing Steven-Johnson Syndrome include:

• Viral infections, including human immunodeficiency virus (HIV), herpes, hepatitis, and pneumonia
• Using certain specific medications
• Having the HLA-B1502 gene, which increases one’s susceptibility to SJS
• A weakened immune system owing to HIV infection, autoimmune disorders, or transplants
• Having Steven-Johnson Syndrome previously
• Family history of the syndrome
• Stevens-Johnson Syndrome maybe aggravated by systemic corticosteroids

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Stevens-Johnson Syndrome? (Etiology)

The causes of Steven-Johnson Syndrome include:

• Certain medications or therapies are known to cause the condition (also known as drug-induced Steven-Johnson Syndrome), and these include:
  • Antibiotics that contain penicillin, sulfa or tetracyclines
  • Pain relievers, such as Advil, Tylenol, or Aleve
  • Seizure medications, such as anticonvulsants
  • Anti-gout medications, such as Allopurinol
  • Barbiturates
  • Phenytoin
  • Radiation therapy
• Infections that include:
  • HIV infection
  • Herpes simplex virus or herpes zoster virus infection
  • Hepatitis viral infection
  • Influenza infection
  • Typhoid infection
• UV light exposure

What are the Signs and Symptoms of Stevens-Johnson Syndrome?

The signs and symptoms of Steven-Johnson Syndrome include:

• Red and blistered, bloody, or crusted lips (chelities), mouth (stomatitis), and genitals (mucosal ulceration)
• Lesions on the hands and feet
• Conjunctivitis: Painful, sticky, red eyes that can result in scars
• Facial or tongue swelling
• Spreading skin rashes, hives
• Extensive skin blistering can be very painful
• Shedding of the skin
• Tender skin patches, that are small-to-large and may peel-off
• High fever
• Swollen lymph glands
• Pneumonia

How is Stevens-Johnson Syndrome Diagnosed?

A diagnosis of Steven-Johnson Syndrome may include:

• Examining the patient’s medical history
• A physical exam looking for the presence of distinctive symptoms of Steven-Johnson Syndrome
• Skin biopsy: A skin biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Stevens-Johnson Syndrome?

The possible complications of Steven-Johnson Syndrome include:

• Secondary infection of the skin (cellulitis)
• Infection of the blood (sepsis) from the skin infection
• Permanent skin and mucosal damage, including scaring and pigment changes
• Eye defects including inflammation and even blindness
• Joint contractures
• Internal organ damage

How is Stevens-Johnson Syndrome Treated?

The treatment for Steven-Johnson Syndrome includes the following measures:

• Immediate admission to the hospital, with severe cases being admitted to the intensive care unit or burns unit to treat extensive skin damage. A ‘burns unit’ usually has the personal and equipment to deal with conditions of extensive skin damage
• Discontinuing medications that could be potential causes of SJS
• Administration of IV fluid and nutrition
• Medications:
  • Antihistamines to reduce itching
  • Antibiotics to treat secondary infections
  • Pain medication, such as morphine
  • Topical steroids to alleviate skin inflammation
• IVIG (Immunoglobulin intravenous) therapy: The immunoglobulins help boost the immune system in individuals with decreased immunity
• Skin grafting (skin transplant)

How can Stevens-Johnson Syndrome be Prevented?

• Currently, there are no prevention methods available for Stevens-Johnson Syndrome
• The disease can recur, if it was caused by a specific medication. Therefore, not taking certain medications can reduce the risk for the condition

What is the Prognosis of Stevens-Johnson Syndrome? (Outcomes/Resolutions)

• Stevens-Johnson Syndrome is a potentially life-threatening condition
• A prompt treatment results in favorable prognosis in majority of individuals. A delay in treatment results in a poor prognosis (high rate of mortality)
• The syndrome has an overall survival rate of 90%
• A recurrence of Stevens-Johnson syndrome is fatal in many cases, because it is more severe the second time around

Additional and Relevant Useful Information for Stevens-Johnson Syndrome:

• Erythema multiforme is an acute, benign skin condition with a sudden onset that usually occurs due to medications, infections, illnesses, or for unknown reasons. Steven-Johnson Syndrome is the most severe form of erythema multiforme.

The following article link will help you understand erythema multiforme:

http://www.dovemed.com/diseases-conditions/erythema-multiforme/