Osszefoglalo. Ket fiatal nobetegnel a valproatrol lamotriginre torteno gyogyszeratallitas soran a 3-4. heten influenzaszeru prodromalis tuneteket kovetoen toxikus epidermalis necrolysis (TEN), mas neven Lyell-szindroma alakult ki. Mindket beteg 5 napja kezdodott bor- es nyalkahartyatunetekkel, kiterjedt hamlevalast okozo hamnekrozissal kerult felvetelre a Debreceni Egyetem Borgyogyaszati Klinikajanak Egesi Intenziv Osztalyara. Multidiszciplinaris szupportiv terapia mellett nagy dozisu szteroid- es immunglobulin-terapiat alkalmaztunk. A 37 eves nobetegnel 3 het utan a korkep fatalis kimenetellel vegzodott. A 19 eves nobeteg tunetei 4 het intenziv terapia utan szovodmenyekkel gyogyultak. A TEN ritka, gyogyszer altal okozott, eletet veszelyezteto, kesoi hiperszenzitivitasi reakcio. Patogeneziseben a gyogyszermolekula, a human leukocytaantigen (HLA) I. osztalyu molekula es a T-sejt-receptor koros interakcioja szerepel. Kezeleseben a legfontosabb a kivalto gyogyszer elhagyasa, valamint az azonnal kezdett komplett szupportiv terapia alkalmazasa. A specifikus kezelest illetoen nincsenek egyseges szakmai iranyelvek. A veszelyes gyogyszerek titralt bevezetese csokkentheti a kialakulo hiperszenzitivitas sulyossagat, ezenfelul a beteg szoros kovetese es az adverz tunetek korai felismerese javithatja a TEN kimenetelet. Orv Hetil. 2020; 161(46): 1959-1965. Summary. After switching from valproate to lamotrigine, on the 3rd-4th weeks, two young female patients developed flu-like prodromal symptoms, followed by the development of toxic epidermal necrolysis (TEN), also known as Lyell syndrome. Both patients were admitted to the Burn Intensive Care Unit of the Department of Dermatology, University of Debrecen with skin and mucosa symptoms; extensive epithelial death and detachment started 5 days earlier. In addition to multidisciplinary supportive treatment, high-dose corticosteroid and immunoglobulin therapy were administered. In the case of the 37-year-old female patient, the disease resulted in a fatal outcome. The 19-year-old patient healed with some sequelae. TEN is a rare, life-threatening delayed-type hypersensitivity reaction caused by drugs. Its pathogenesis involves an interaction between small-molecule drug, human leukocyte antigen class I molecule and T-cell receptor. The most important treatment is immediate withdrawal of potentially causative drugs and prompt application of supportive therapy. There is no standard guidance on specific treatment. Slow dose escalation of dangerous drugs can be beneficial in avoiding severe reactions, furthermore, close patient follow-up and early detection of the possible adverse reactions contribute to a more favourable outcome of TEN. Orv Hetil. 2020; 161(46): 1959-1965.
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