Механізми етіопатогенезу, клініки та сучасні підходи до лікування синдрому стівенса-джонсона та токсичного епідермального некролізу

Abstract

Abstract. This article contains descriptive data on the etiology, pathogenesis, clinical presentation, diagnostics and treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). SJS and TEN are relatively rare conditions, however, due to their severity, lethality and long-term negative consequences it is not only necessary to understand their etiopathogenesis clearly, but it’s also crucial to be alert to the patient’s allergic anamnesis for timely assessment of their condition and diagnosis with following adequate therapy. Prevalence of these diseases is from 0.4 to 1.4 cases per million persons per year, total lethality is approximately — 20 %. Development of these diseases is related to genetic predisposition of the human leukocyte antigen system (HLA). Medications are the most common triggers and play an important role in their onset, other factors, including infections, can also significantly affect the risk of their inception. In the present day, the SCORTEN scale is used to assess the severity of SJS and TEN. General principles of patients’ curation are based on the multidisciplinary approach, early diagnosis, discontinuation of trigger medications that could possibly cause SJS and TEN. In case when SJS/TEN were caused by infections, patient receives appropriate etiotropic therapy to eliminate the infection. The complex treatment algorithm must ensure hemodynamic stability, pain reduction, detoxification, local treatment, elimination of concomitant infections, use of anti-inflammatory and various immunotropic agents.