Among severe drug reactions in children, besides Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), a specific form of hypersensitivity syndrome which is nowadays known as "drug reaction with eosinophilia and systemic symptoms" (DRESS) has to be mentioned. Whereas SJS/TEN is considered one reaction entity of different severity, DRESS has to be distinguished from SJS/TEN but also from other severe exanthems due to multiorgan involvement. Although SJS/TEN is generally referred to as a drug reaction, only about 75% of all cases are actually caused by medications and in children it is only about 50%. After a clear diagnosis has been made, specific therapeutic measures can follow, of which withdrawal of the inducing agent plays a key role, but further treatments differ substantially. In order to identify and withdraw the inducing agent, a detailed and thorough medication history must be obtained. Highly suspected drugs of SJS/TEN in children include, among others, antibacterial sulfonamides and various antiepileptics. DRESS in children and adolescents is also frequently induced by antiepileptics, but also by sulfonamides and minocycline. In contrast to adults, allopurinol is rarely found to be culprit in both conditions. Supportive therapy including appropriate topical treatments, pain therapy, ophthalmologic consultations, etc. is the gold standard in SJS/TEN, but a short-term immunomodulating therapy with cyclosporine A has shown very promising results in recent studies. In DRESS, however, systemic treatment with glucocorticosteroids slowly tapered over a longer period of time is recommended.
Keywords: Drug reaction with eosinophilia and systemic symptoms; Risk factors; Stevens-Johnson syndrome; Therapeutic management; Toxic epidermal necrolysis.