- Toxic epidermal necrolysis (TEN) is a rare, life-threatening disease with a high mortality rate. It is linked to drug toxicity and characterized by epidermal necrolysis with mucositis and conjunctivitis. Treatment is not established due to the unknown pathogenesis and lack of randomized clinical trials. It is mostly based on withdrawal of the culprit drug and symptom-related approach. The role of corticosteroids and plasmapheresis in the disease treatment remains controversial. We present two patients with severe TEN (both with >80% body skin surface involvement) treated unsuccessfully with corticosteroids followed by plasmapheresis. Plasmapheresis led to prompt improvement, with extensive reepithealization of the skin, and eventually total recovery of both patients. In severe TEN unresponsive to corticosteroids, treatment with plasmapheresis should be considered.