Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis. Cases with less than 10 percent epidermal involvement are classified as SJS; those with 30 percent or more involvement are classified as TEN; cases with 10 to 30 percent involvement are considered overlap SJS/TEN. However, we use the term "SJS/TEN" to refer collectively to SJS, TEN, and SJS/TEN overlap syndrome.
Medications are the leading trigger of SJS/TEN in both adults and children. Allopurinol, aromatic anticonvulsants, antibacterial sulfonamides, and "oxicam" NSAIDS are most commonly implicated. Mycoplasma pneumoniae and cytomegalovirus infection are the next most common trigger of SJS/TEN, particularly in children.
Risk factors for SJS/TEN include HIV infection, genetic factors, concomitant viral infections, underlying autoimmune diseases, and possibly physical factors.
SJS/TEN begins with a prodrome of fever and influenza-like symptoms one to three days before the development of mucocutaneous and skin lesions. The cutaneous eruption typically starts with ill-defined, coalescing erythematous macules with atypical target lesions. As the disease progresses, vesicles and bullae form and within days the skin begins to slough. Mucosal involvement occurs in approximately 90 percent of cases of SJS/TEN and can precede or follow the skin eruption.
In severe cases with extensive skin detachment, acute complications may include massive loss of fluids and electrolyte imbalance, hypovolemic shock with renal failure, bacteremia, insulin resistance, hypercatabolic state, and multiple organ dysfunction syndrome.
The diagnosis of SJS/TEN is based upon clinical and histologic findings in a patient with a history of antecedent drug exposure or illness. Histologic findings on skin biopsy are supportive, but not independently diagnostic.
Patients suspected to have Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) should be admitted to the hospital. The severity and prognosis of the disease should be rapidly determined by using the SCORTEN score to define the appropriate medical setting for management.
For patients with extensive skin detachment and/or a SCORTEN score ≥2, we suggest transfer to intensive therapy or burn unit if possible (Grade 2C).
Early recognition and immediate withdrawal of any potentially causative agents are critical first steps in the management of SJS/TEN.
Supportive care is the mainstay of treatment and includes wound care, fluid and electrolyte management, nutritional support, ocular care, temperature management, pain control, and monitoring or treatment of superinfections.
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.
Since sepsis is the major cause of death, infection control measures, including sterile handling, topical antiseptic 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.
Beyond supportive care, there is insufficient evidence to establish the benefit of any adjunctive therapies. Systemic corticosteroids and intravenous gammaglobulin (IVIG) are commonly used at some centers. Our approach is described below.
We suggest NOT using systemic corticosteroids for SJS/TEN (Grade 2C). However, for patients with limited skin detachment and no obvious contraindications to systemic corticosteroids, a short course of moderate to high dose systemic corticosteroids (eg, prednisone 1 to 2 mg/kg per day for three to five days) may be given early in the course of the disease (ie, within 24 to 48 hours of symptom onset).
We suggest NOT using IVIG for SJS/TEN (Grade 2C). However, if a decision is made to use IVIG for individual patients with severe disease, a dose of 1 g/kg per day may be given for three consecutive days (total dose 3 g/kg) early in the course of the disease (ie, within 24 to 48 hours of symptom onset).
The overall mortality rate among patients with SJS/TEN is approximately 25 percent, ranging from approximately 10 percent for SJS to more than 40 percent for TEN. Sepsis, acute respiratory distress syndrome, and multiple organ failure are the most common causes of in-hospital death. Long-term sequelae involving the skin and eyes are common among survivors.