Why so long to diagnosis?
We suspected that the diagnosis was missed during our patient’s earlier ED visits because she initially had nonspecific symptoms and did not have mucosal involvement early on. Further complicating matters: She went to multiple EDs.
Labwork is of limited value
No laboratory values or pathologic tests are pathognomonic for SJS/TEN. Anemia and lymphopenia are possible findings, and neutropenia is an indicator for a poor prognosis.8 Elevated liver enzymes are common, reaching levels approximately 2 to 3 times the upper limit of normal.8 All of these laboratory findings become increasingly more likely as BSA involved increases.8 Our patient’s laboratory values were unremarkable.
Tx: Discontinue drug, replace fluids
This syndrome is potentially deadly and must be treated as an emergency as soon as it is recognized. The mortality rate is 1% to 3% for SJS and 25% to 35% for TEN.9
Discontinuing the causative agent is the first step in treatment. In our patient’s case, Bactrim had been discontinued before she came to our ED. The next step, ideally, is to transfer the patient to a burn unit where she can receive the same type of supportive care burn victims require. Diligent wound care, fluid replacement, electrolyte monitoring, and raised ambient temperature are vital elements in proper care. The patient should also be evaluated throughout the treatment period for any ocular involvement, such as conjunctivitis, keratitis, or severe dryness.10
As is the case with burn victims, the major risks are secondary infection and sepsis. Skin, blood, and access-line cultures should be gathered throughout the hospitalization to evaluate for infection. Some authorities believe glucocorticoids are helpful in children with SJS/TEN, but the adverse effects are sufficiently significant that the risks may outweigh the benefits. In adults, the evidence favors the use of glucocorticoids in SJS, but not in TEN, where the increased risk of sepsis following immunosuppression may outweigh the benefits.11
Intravenous immunoglobulin (IVIG) may benefit both children and adults, and the benefit appears to outweigh risks associated with this therapy.12 Plasmaphoresis to remove toxic metabolites from the circulation is an additional treatment option, but there is no strong evidence to support this approach.13
A positive outcome for our patient
Our patient was transferred to a facility with an inpatient burn unit and a consulting dermatology service. She was maintained on steroids, but neither plasmapheresis nor IVIG was necessary.
She was discharged to her home with prescriptions for topical emollients and an oral steroid solution for her mouth irritation. Systemic steroids were not continued, as the relatively small area of desquamation made this unnecessary.
Staying safe hinges on education. Our patient was advised that going forward, she needed to avoid Bactrim and any other sulfa drugs. She was told that when medications are prescribed for her in the future, she must consult with the pharmacist to make sure sulfonamides are not included.11
CORRESPONDENCE Bryan Cairns, MD, 2123 Auburn Avenue, Cincinnati, OH 45219; Bryancairns@gmail.com