[Lyell's acute epidermal necrosis or bullous erythroderma with Debré-Lamy-Lamotte epidermolysis].
キーワード
概要
Acute epidermal necrosis is an exceptionally serious condition whose onset, which is sometimes preceded by a few prodomes, is marked by an eruption mainly around the orifices and rapidly extending to the whole of the tegument. Epidermal necrosis results in extensive shedding of the skin and within the space of a few hours gives the patient the appearance of having been scalded. In adults, drugs and in particular certain combinations of drugs, are responsible; the drugs mainly involved are the sulfonamides, especially delayed action sulfonamides, derivatives of butazolidine, Barbiturates, Phenindione and Penicillin. In children, on the other hand, acute epidermal necrosis is usually secondary to infection by a phase 2 coagulase positive staphylococcus aureus, this staphylococcus secreting an exotoxin which appears to act by breaking up the desmosomes. Treatment mainly consists of emergency resuscitation.