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Pistone G. 1, La Vecchia M. 1, Argo A. 2, Barranco R. 2
1 Dipartimento di Dermatologia, Università degli Studi di Palermo, Palermo, Italy;
2 Sezione di Medicina Legale, Dipartimento di Biopatologia e Biotecnologie Mediche e Forensi, Università degli Studi di Palermo, Palermo, Italy
Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare adverse drug reactions that firstly involve the skin and mucous membranes. They are considered as two clinical variants of the same pathological potentially which can be fatal and differ depending on the extent of mucocutaneous involvement. The role of specific HLA haplotypes is essential in the etiologic consideration as well as certain drugs including ACE inhibitors, allopurinol and carbamazepine. Although the pathogenetic point of view has highlighted the predominant role of Fas / FasL and granzyme B in the onset of keratinocyte apoptosis, recent studies indicate the granulosina as a key element in the onset of SJS / TEN. The clinical picture is characterized by hemorrhagic erosions, erythema and epidermal detachment greater or lesser extent. The essentially clinical and histopathological diagnosis, requires a certain timeliness in order to allow a suitable treatment and avoid the onset of the patient. The SJS / TEN due to acute complications, which can be chronic and often fatal, is considered an issue of medicolegal interest. The possibility of tracing any of the medical professional liability profiles is difficult to perform because the morbid picture is unpredictable and not preventable and because of the presence of possible confounding factors, which may make us unable to trace the causal relationship and determine the negligent conduct of each health care professional.