Treatment And Prognosis for Stevens-Johnson Syndrome

Early diagnosis and recognition with prompt withdrawal of all suspected potential causative drugs are essential for a favorable outcome. Morbidity and mortality decrease if the culprit drug is withdrawn no later than the day when blisters or erosions first occurred. No difference was seen for drugs with long half-lives. The patient must be transferred to an intensive care unit or a burn center. Rapid referral reduces risk of infection, mortality rate and period of hospitalization. Supportive care is the mainstay of treatment. Intervention should include intravenous fluid and electrolyte replacement, environmental temperature control, careful and aseptic handling, early initiation of enteral nutrition by nasogastric tube and pain and anxiety control. Many remedies have been proposed for topical care. Silver-sulfadiazine (silverol) should be avoided because it contains sulfa which is one of the culprit drugs capable of inducing SJS-TEN.

Systemic Corticosteroids. Systemic steroid therapy has been the accepted treatment for SJS-TEN for years. It is believed to suppress the intensity and the extension of the necrolytic process in the skin as well as in internal organs.

Conversely, the use of steroids has also almost become a contraindicated mode of therapy. They were regarded as being hazardous, owing to reports of iatrogenic decrease of host resistance, increase of morbidity and complications, prolonged recovery and reduced survival, following their use.

Parallel to the change in regard to steroid treatment in SJS-TEN, the management of this severe disease was shifted to specialized burn centers and was taken over by nondermatologists, mostly surgeons, who sometimes erroneously regarded SJS-TEN and burns as similar entities. However, in terms of etiology and pathogenesis, a burn is a one-time acute event that affects the skin from the outside, whereas SJS-TEN is a more complex, probably immune (T lymphocyte)-mediated process that reaches the skin from within. The disease process continues to progress over a period of several days after first appearance.

Today, in the absence of clinical control trials, most authors believe that systemic steroids are of unproven benefit in early forms of and are clearly harmful in advanced SJS-TEN.

Intravenous Immunoglobulin. As mentioned previously, the results of IVIg treatment are not uniform in all reports. It should be avoided in patients with renal failure, as increased mortality has been observed. Thus, currently intravenous immune globulin is not considered part of the standard of care for TEN.

In conclusion, optimal treatment for the SJS-TEN spectrum remains to be clarified. To date, no specific treatment has been proven to be beneficial for SJS-TEN. The best management is early recognition, prompt withdrawal of causative drugs and supportive care.

Prognosis
In order to rank severity and predict prognosis in TEN patients, the severity-of-illness score (SCORTEN), was developed. The score is a mathematical tool consisting of the sum of seven clinical variables (age, history of malignancy, heart rate, initial epidermal detachment, admission blood urea nitrogen, glucose levels and serum bicarbonate) as prognostic factors.

The SJS-TEN spectrum is an acute illness with potentially life-threatening complications. Reported mortality rates are 5% with SJS, 10-15% with overlap forms and 30-35% with TEN. Most deaths in patients with TEN are a result of sepsis-induced organ failure.

TEN might also result in late complications, such as transitory hyper- and/or hypopigmentation, scarring primarily due to pressure or secondary infections, alopecia, anonychia and sicca-like syndrome. Phimosis and vaginal synechiae may also be present. Ocular sequelae affecting up to 40% of the survivors may cause corneal lesions, which may result in severe impairment of vision. Therefore, intensive ophthalmologic follow-up and treatment is mandatory in patients with the SJS-TEN spectrum.

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