distinguish from SJS/TEN In those cases, initiation of treatment for SJS/TEN is prudent while awaiting results from an infectious workup The differential diagnosis for SJS/TEN includes EM, staphylococcal scalded skin syndrome (SSSS), and Kawasaki disease As noted above, although SJS/TEN can have targetoid lesions, these are not the classic target lesions seen in EM Additionally, SJS/TEN usually begins on the face and trunk, rather than the extremities as in EM Likewise, although EM can have mucous membrane involvement, it does not involve two or more mucous membranes SSSS affects the superficial epidermis, resulting in superficial desquamation rather than the full-thickness epidermal necrosis seen with SJS/TEN SSSS also spares the oral mucosa, while SJS/TEN affects the oral mucosa In contrast, Kawasaki disease often has a mucosal involvement, with conjunctivitis, strawberry tongue, and dry and cracked lips However, the degree of involvement is not as severe as that seen in SJS/TEN, which may frequently consist of widespread erosions within the mouth and thick hemorrhagic crust on the lips Similar to other drug reactions, the most important step in managing SJS/TEN is stopping the causative medication Wound care is critical to decrease the risk of complications, including infection and scarring Petrolatum gauze or plain petrolatum should be liberally used to prevent scarring in all affected areas, including the lips, genitals, and anus Ophthalmology and urology should be consulted if there is suspected ocular or urethral involvement The skin should be examined daily, and signs of infection should prompt aggressive treatment because the primary cause of mortality is infection Mortality from SJS/TEN can be as high as 30%, with mortality increasing proportionally to the amount of body surface area involved Pain management and nutritional support may need to be provided parenterally since oral involvement may limit oral intake Regarding medical treatment, there is no consensus as to whether systemic steroids or intravenous immunoglobulins (IVIG) have benefit in this condition Systemic steroids have generally fallen out of favor because of the increased risk of infection and delayed wound healing IVIG is often used because it is thought to block apoptosis signaling pathways When used, IVIG is given at 0.5 to g/kg/day for to days to reach a total dose of to g/kg Recently, there is evidence that tumor necrosis factor alpha (TNFa) inhibitors may be effective for the treatment of SJS/TEN, though there have only been a few case reports of its use in pediatrics Suggested Readings and Key References