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An 8-year-old patient is admitted with severe mucocutaneous erythema and fever. He looks acutely ill, with haemorrhagic crusting on his lips and complains of burning pain. He is diagnosed with Stevens-Johnson Syndrome (SJS) and supportive measures are commenced. You wonder if in this scenario systemic steroids would be useful. You decide to look at the evidence.
Structured clinical question
In children with SJS (patient) do systemic steroids (intervention) reduce morbidity or mortality (outcome)?
Search strategy and outcome
PubMed and Scopus databases were searched using search terms: Stevens-Johnson syndrome AND steroids AND (management OR treatment OR therapy).
Inclusion criteria were: children included in study population, article in English, evidence level 2 or higher (using Oxford Centre for Evidence-based Medicine criteria1).
Altogether, 189 papers were found in Pubmed, of which 186 were irrelevant or of insufficient quality. Three hundred and ten papers were found in Scopus, of which one was a further relevant paper. All relevant papers are summarised in table 1.
SJS (also known as Lyell's disease) and toxic epidermal necrolysis (TEN) are severe, sometimes life-threatening skin reactions that present with mucocutaneous epidermal detachment.2 Classically considered drug reactions, other aetiological factors include viral infections, M pneumoniae, vaccines and some neoplastic and autoimmune conditions.3
There are no universally accepted diagnostic criteria and confusion over nomenclature between SJS, TEN and erythema multiforme has been only partially resolved. Bastuji-Garin et al in 19934 and Roujeau in 19945 published a consensus definition distinguishing erythema multiforme as a distinct entity, with SJS and TEN variants of severity in a spectrum of the same disease process. Estimates of incidence vary widely, perhaps reflecting the use of varying classification systems, from 0.4 to 1.2 and 1.2 to 6 …