SUMMARY
Stevens-Johnson syndrome and toxic epidermal necrolysis are rare life-threatening diseases that manifest with bullae formation and denudation of the skin and mucosa. They are most often a consequence of an immune-mediated drug reaction, rarely due to other causes. After discontinuation of the culprit drug and treatment of bacterial infection, if proven as a cause of the disease, local eye, skin, and mucosal therapy are prescribed. The drugs most often used in the systemic treatment are glucocorticosteroids (GCS) and intravenous immunoglobulins (IVIG). The cooperation between various specialists is of crucial importance.We present a case series of patients treated at the Department of Alergology, Rheumatology and Clinical Immunology, University Children’s Hospital Ljubljana, Slovenia, and a review of the literature. From 2011 – 2019 we treated six children with SJS/TEN. In four children the disease was associated with a drug, in one child with infection with Mycoplasma pneumoniae and in one child the cause of the disease was not identified. Four children were treated with GCS and IVIG, the child with Mycoplasma pneumoniae infection was treated with azithromycin and IVIG, one child was treated only with local therapy. The outcome of the disease was good in all patients, without late sequelae.