Toxic epidermal necrolysis (TEN) is a rare but life-threatening mucocutaneous adverse drug reaction. The disease is characterized by a specific and extensive destruction of the epidermis and mucosal epithelia, particularly of the mouth, genitalia and eyes. The TEN pathomechanism is probably initiated by a toxic drug metabolism inside keratinocytes, leading to a self-activation of apoptosis and necrosis. These events are boosted by additional effects of T lymphocytes and macrophages. At present there is still a lack of validated mainstay treatment for TEN. However, a few treatment modalities have been reported to halt TEN progression in some patients.