Toxic Epidermal Necrolysis
Florence Castelain and Philippe Humbert
Affiliation: Department of Dermatology, Hopital Saint Jacques, 25030 Besancon Cedex, France.
Keywords: Toxic epidermal necrolysis, Lyell, Stevens-Johnson, SCORTEN, immunoglobulin, intravenous, paraneoplastic, pemphigus, mucocutaneous, bullous, erythematosus
Toxic epidermal necrolysis (TEN) is a severe mucocutaneous drug-induced syndrome that causes massive
keratinocyte apoptosis and therefore hydro-electrolytic disorders and systemic infection. TEN approximately affects one
to two cases per million per year. Mortality rate may reach thirty percent of cases. Thus, TEN constitutes a therapeutic
emergency at diagnosis. Typically, clinical examination shows a mucocutaneous detachment involving more than thirty
percent of body area. Definitive diagnosis is made on cutaneous biopsy with histological exam that shows the blister of
necrotic keratinocytes. Main differential diagnosis are acute staphylococcus epidermis, acute generalized exanthematous
pustulosis, linear IgA bullous dermatosis, paraneoplastic pemphigus, bullous fixed pigmented erythema, acute lupus
erythematosus. In the early days, SCORTEN gives a good estimation and is now widely used as prognostic score. Drugs
are generally considered as the main etiology of TEN but in some cases bacterial or viral infections could be involved.
Physiopathology remains unclear even if recent advances have reported the possible implication of immune pathways
based on activation of T and NK cells. Treatment of TEN requires to be instituted as soon as the diagnosis is made and the
patient is preferentially referred to a specialized unit. Supportive care consist of covering areas of cutaneous detachment.
No other therapy has demonstrated its efficiency, but high-dose intravenous immunoglobulin might improve the
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