Stevens-Johnson syndrome and toxic epidermal necrolysis are rare, life-threatening, cutaneous drug reactions. Medications are the most common cause, although an infection may be responsible. A link between genetics and certain medications has been established. Clinical diagnosis should be confirmed with biopsy. When the area of epidermal detachment approaches 30%, burn center care is advisable. An ophthalmologist should be consulted to optimize ocular care. Pharmacologic interruption has been sought but there is little consensus on the most appropriate agent and no high-quality studies have been conducted to demonstrate if any of these agents lead to improved survival.
Although patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now routinely referred to burn centers for definitive care, not all cases of SJS or even SJS-TEN overlap automatically warrant burn center admission. However, the more extensive the degree of exfoliation (especially in TEN), the greater is the need for and benefit from providing care in a burn center setting.
Diagnosis of SJS and TEN relies on careful documentation of systemic, cutaneous, and mucosal features obtained from a detailed history and physical examination, combined with histologic confirmation by biopsy.
The treatment bundle in the burn center should include cessation of the causative medication; careful airway assessment and protection, if indicated; directed (rather than routine) fluid replacement for hypovolemia; early enteral nutrition; wound coverage with skin substitutes; urgent ophthalmologic consultation; careful surveillance for infection; avoidance of prophylactic antibiotics; and consideration of a pharmacologic intervention to attempt to halt the disease process.
Pharmacologic interventions to halt the disease process have not been examined through large high-quality studies and consensus on use of these agents is lacking.