A systematic review of treatment of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children

Abstract

Stevens-Johnson (SJS) and Toxic Epidermal Necrolysis (TEN) are two uncommon mucocutaneous diseases usually considered as severe drug reactions and are characterized by different grades of epidermal necrosis. Several treatment modalities have been proposed with variable results but the lack of controlled studies makes difficult to analyze them objectively especially in children. All publications describing management for SJS and TEN in children were searched in MEDLINE, EMBASE, and the Cochrane Library. Reports included were divided in two categories: A, studies with 5 or more patients and observational studies; and B, reports with less than 5 patients. A formal meta-analysis was not feasible. Description was made using central tendency measures. From 1389 references only 31 references with a total of 128 cases were included, 88 category A and 40 category B. The 4 main treatment modalities were: intravenous immunoglobulin (IVIG), steroids (prednisolone, methylprednisolone, dexamethasone), dressings with or without surgical debridement, and support treatment alone. Miscellaneous treatments: Of 12 patients, 3 received ulinastatin, 4 patients plasmapheresis, 2 patients IV pentoxifylline and the last three patients received different treatment each (cyclosporine, methylprednisone/G-CSF and methylprednisolone/IVIG). Patients receiving IVIG and steroids showed similar findings while patients treated with dressing and support treatment alone, reported both longer time to achieve remission and hospitalization stays and appear to be associated with more complications and deaths. There is scant quality literature about management of SJS and TEN in children. Steroids and IVIG seem to improve the outcome of SJS and TEN patients but results from different reports are variable. Patients treated only with care support seem to have higher morbidity and mortality. Further studies are necessary to define optimal management.