- Early recognition and immediate withdrawal of any potentially causative agents are critical first steps in the management of SJS/TEN.
- Multiple specialists should be involved in the care of patients with SJS/TEN when possible, including experts in critical care, plastic surgery, dermatology, infectious disease, ophthalmology, and nutrition.
- For patients with extensive desquamation, we suggest transfer to a burn unit if possible (Grade 2C).
- The optimal approach to wound care has not been determined. Success has been reported with both repeated debridement of exfoliating skin, and "anti-shear" wound care, in which the necrotic skin is left in place to act as a biologic dressing.
- Sepsis is the major cause of death. Sterile handling, infection control measures, topical antibiotic agents, and surveillance cultures of possible sites of superinfection are important components of prevention. Prophylactic systemic antibiotics are not utilized by the majority of burn centers, although antimicrobials should be administered at the first sign of infection, and choice of agent should be guided by specific culture data.
- Supportive care should be the primary focus of management of SJS/TEN. Beyond this, there is insufficient evidence to establish the benefit of any adjunctive therapies (table 1). Systemic glucocorticoids and intravenous gammaglobulin (IVIG) are commonly used at many centers, although not all. Our approach is described below.
- For children (aged 16 years and younger) with SJS, we suggest NOT administering glucocorticoids (Grade 2C).
- For adult patients with mild to moderate SJS, in whom the diagnosis has been made within a few days of symptom onset, we suggest high dose, short-term systemic glucocorticoids (Grade 2C). We typically use prednisone, 2 mg per kg daily or an equivalent amount of prednisolone or methylprednisolone, initiated as soon as possible after diagnosis. Glucocorticoids are discontinued after four to seven days or at the first sign of infection.
- For pediatric and adult patients with severe SJS and TEN, we suggest IVIG (Grade 2C). We administer a dose of 1 gram/kg daily for three consecutive days. We do not administer glucocorticoids, due to concern about increasing the risk of sepsis, or employ plasmapheresis in the treatment of TEN.
- The mortality of SJS is 1 to 3 percent, while the mortality of TEN ranges from 25 to 35 percent. Predictors of mortality include older age at onset and greater extent of skin involvement. Long-term sequelae of the skin and eyes are common among survivors.
Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae | ||
Whitney A High, MD Milton H Nirken, MD | UpToDate |
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