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Stevens Johnson Syndrome

Stevens Johnson Syndrome (SJS) is a rare and potentially fatal medical Stevens Johnson Syndromedisorder involving the skin and mucous membranes. Technically, SJS is an immune-complex-mediated hypersensitivity condition, which basically means it occurs due to an allergic reaction. While SJS is usually caused by an allergic reaction to drugs, it can also be caused by a reaction to an infection or illness.

When caused by an adverse drug reaction, SJS usually develops one to three weeks after taking the causative drug. Initially, patients develop a rash. Then, a few days later, the rash turns into skin lesions. The skin lesions tend to coalesce, creating large blisters.

A hallmark of SJS is epidermal detachment, meaning the infected patient’s skin starts to literally fall off in large sheets. Often, the denuded area looks like an extensive burn. Due to this horrific side effect, Stevens Johnson Syndrome can be extremely morbid, possibly resulting in

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permanent scarring, blindness, or even death.
 
SJS Background
The term “Stevens Johnson Syndrome� dates back to 1922 when two U.S. pediatricians named Albert M. Stevens and Frank C. Johnson documented dermatological eruptions, mucosal and buccal ulcerations, high fever, and conjunctivitis (pink eye) in two young boys. In 1969, another physician wrote about two pediatric patients with “scalded skin� and massive epidermis (skin) loss, calling the condition “Toxic Epidermal Necrolysis� (TEN).

Today, most authors and experts agree that SJS and TEN are both manifestations of the condition known as erythema multiforme, which is another term for a skin disorder caused by a reaction to a drug, infection or illness. Additionally, SJS and TEN are both characterized by identical clinical signs and symptoms, identical treatment, and identical prognosis. For those reasons, some refer to SJS and TEN interchangeably and others simply refer to Stevens Johnson Syndrome as “SJS/TEN.�

SJS Statistics
The incidence of SJS is estimated to be two to six cases per million people per year. The illness affects all ages and races. In addition, SJS occurs in both males and females. The mortality rate in adults is estimated at up to 30%. However, children seem to recover from SJS more frequently, with an estimated mortality rate between 3% and 5%.


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