Toxic epidermal necrolysis is the prototype of a proapoptotic disease characterized by system CD95 dysrregulation. Drugs constitute the main antigenic triggers. Hystopatologically it is characterized by epidermis detachment and necrosis with apoptotic keratinocytes. Clinical presentation includes erithematous-ampullous lesions in the skin and mucous membranes. It is associated with serious complications such as severe sepsis and septic shock. The management in the intensive care unit includes support treatment and specific treatment with immunoglobulins that alter disease course. Recombinant activated Factor VII is effective to control the associated microvascular haemorraghe.

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