Clinical features of Stevens-Johnson syndrome and toxic epidermal necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but these conditions are associated with high mortality. There have been few reports of SJS and TEN in children. The aim of this study was to evaluate the clinical features and outcomes of SJS and TEN in a group of Japanese children.

We retrospectively reviewed pediatric cases of SJS and TEN, from 2000 to 2015.

We identified 12 pediatric cases of SJS and three of TEN. Six (all SJS) were caused by infection, and eight of the cases (SJS, n = 5; TEN, n = 3) were drug induced. Respiratory complications were the most common in terms of organ involvement, followed by hepatitis and gastrointestinal symptoms. Thirteen patients were treated with systemic corticosteroids, and two patients were treated with supportive therapy only. Concomitant with corticosteroid, four patients were given i.v. immunoglobulin. One patient with severe TEN was treated with systemic corticosteroids combined with plasmapheresis and cyclosporine. None of the present patients died. One patient with TEN had severe sequelae, with bronchiolitis obliterans and ocular involvement.

SJS/TEN are rare, but are associated with severe complications. General pediatricians need to have up-to-date information regarding these conditions. The present study provides insights into the confirmation of the risk of SJS/TEN as well as the treatment of these diseases.