Pediatric emergency medicine trisk 1319 1319
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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 1
g/kg/day for 2 to 4 days to reach a total dose of 2 to 4 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.
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