Stephens Johnson Syndrome

Stevens-Johnson Syndrome is a rare hypersensitivity disorder in which the skin and mucous membranes react severely to some sort of infection or stimulus. This disease is most commonly identified by the rash that leads to loss of skin, and is almost always identified as an emergency. If a patient with SJS is left untreated, he suffers serious risk of death.

Causes

• According to Consultant for Pediatricians, Stephens-Johnson Syndrome seems to be caused by an adverse immune system reaction. Almost any medication can induce it, including over-the-counter drugs. Viral infections such as herpes, the stomach flu, and mumps can cause it as well, and in some cases the inducing factor may be traced back to cancer, thought that is not so common.

Symptoms

• The patient may experience facial swelling, hives, and skin blistering. A red or purple rash develops, and typically as the disease evolves the skin literally sloughs off. Flu-like symptoms occur several days before the rash, including fever, sore throat, etc. Research indicates that only 1-5% of patients die from this disease, though for its more serious form, known as toxic epidermal necrolysis, mortality rate can be as high as 25-35%.

Demographics

• Dr. Parillo's research indicates that SJS affects predominantly Caucasians, with a male to female ratio of 2:1. Most of the patients are somewhere between their 20s and 40s, but cases have been reported for children as young as 3 months old.

Complications

• SJS can lead to several life-threatening complications, including secondary skin infection, eye problems, and damage to the internal organs. Skin infection (cellulitis) is not just painful; it can lead to infection of the membranes surrounding the brain (meningitis) or severe bacterial infection within the bloodstream (sepsis). The rash often causes inflammation in the eye, and in some cases the disease will create lesions in the internal organs as well as the skin. These are all severe complications that should be attended to immediately, if possible.

Treatment

• There is currently no set treatment for SJS as a disease, only for the symptoms. Skin lesions are treated like severe burns while fluids and nutrients are replenished through IV. Complications are treated accordingly. Meanwhile, the doctor questions the patient and checks his medical history to determine the underlying cause. If, for example, it had been an adverse reaction to a certain antibiotic, the patient should slowly begin recovering on his own once he is off that medication. After he has fully recovered, he should stay away from that medication for the rest of his life.

Misconceptions

• People often consider Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis to be interchangeable names. This is true in the sense that they both refer to the same disease. However, there is a clear difference between the two. SJS refers to the disease when less than 10% of body surface area is involved. TEN refers to the disease when 30% or more of the body's surface area is involved. In a nutshell, SJS is the milder form of TEN, and the names refer to varying degrees of severity.