Stevens-Johnson syndrome

Stevens-Johnson syndrome (SJS) is a life-threatening condition affecting the skin, in which due to cell death the epidermis separates from the dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers.

Classification
There is agreement in the medical literature that Stevens-Johnson syndrome can be considered a milder form of toxic epidermal necrolysis (TEN). However, there is debate whether it falls on a spectrum of disease that includes erythema multiforme and erythema multiforme major (also known as erythema multiforme majus).

Epidemiology
SJS is a rare condition, with a reported incidence of around one case per million people per year.

Causes
SJS can be caused by infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), allergic reactions to drugs, (Dicloflex, fluconazole, valdecoxib, penicillins, barbiturates, sulfas, phenytoin, Modafinil, lamotrigine, nevirapine, Ibuprofen, ethosuximide, carbamazepine) , malignancy (carcinomas and lymphomas), or idiopathic factors (up to 50% of the time). SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage.

Although Stevens Johnson Syndrome may be caused by viral infections or malignancies, severe allergic reactions to medication is the leading cause. Medications that have traditionally been known to lead to Stevens Johnson Syndrome, Erythema multiforme, and Lyell's Syndrome (Toxic Epidermal Necrolysis) include;


 * sulfonamides (antibiotics)
 * penicillin's (antibiotics)
 * barbiturates (sedatives)
 * phenytoin - Dilantin (anticonvulsant)

Treatment
Discontinue all medications, particularly those known to cause SJS reactions. Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment (2002). Treatment with corticosteroids is controversial since it might aggravate the condition or increase risk of secondary infections. Other agents have been used, including cyclophosphamide and cyclosporine, but none have exhibited much therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids leading to corneal vascularization and impaired vision, as well as a host of other ocular problems. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital.

Prognosis
SJS proper (with less than 10% of body surface area involved) has the mortality rate of around 5%. The risk for death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account. Other outcomes include organ damage and blindness.

Eponym
It is named for Dr. Albert Mason Stevens and Dr. Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in 1922.