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Minerva Medicolegale 2002 June;122(2): 95-106


language: Italian

Lyell’s syndrome. New therapeutic strategies and medicolegal implications

Cortellini M., Bollero D., Stella M., Magliacani G.


Lyell's syndrome or toxic epidermic necrolysis (TEN) is a rare cutaneous pathology with a fatal prognosis. It is exfoliative in appearance and drug induced and over the past few years it has been increasingly treated by plastic surgeons specialising in burns, given that its clinical onset is similar to an extensive partial thickness burn. It has been recently demonstrated that TEN is caused by an alteration in keratinocyte apoptosis control, mediated by the interaction between Fas ''suicide'' receptors on the membrane of these cells and their respective ligand (Fas ligand, FasL or CD95L). The hyper regulation of Fas L expression is the key to the selective destruction of keratinocytes during TEN. Numerous systemic treatments were used in the past in an attempt to stop the dermo-epidermal detachment with divergent results, but with no significant improvement in patient survival rates. However, a treatment based on the use of i.v. immunoglobulins (IVIG) has recently been introduced with excellent preliminary results. The strength of this therapy can be attributed to the inhibition of the Fas-mediated death of keratinocytes through natural Fas-blocking antibodies contained in the preparations with human immunoglobulins. This study reports our experience using IVIG in the treatment of Lyell's syndrome and analyses the difficulty of defining the correct forensic and legal responsibility of the medical personnel who introduced the therapy that triggered the syndrome. A complete recovery was obtained on average in 12 days (range 7-17 days). In terms of complications, 3 of the 7 surviving patients (8 were treated in all) developed severe respiratory insufficiency requiring mechanical ventilatory support and 1 patient developed acute renal failure requiring hemodialysis. An analysis of the pre-syndrome treatment protocols highlights the difficulty of attributing a catalytic role to any one drug, given that many patients were receiving multiple treatment that had continued for a long time. The unforeseeable nature of the disease and the impossibility of prevention places the doctor who prescribed the drug responsible for triggering this reaction in a position of non-imputability given that he could not have foreseen such a violent reaction to a therapeutic choice specifically targeted to match the patient's conditions.

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