Toxic Epidermal Necrolysis (Lyell's Syndrome): A Report on Four Cases with Three Deaths

Abstract

An account is given of 4 cases of toxic epidermal necrolysis, 3 with lethal course. Three of the cases were of primary type, the first caused by Salazopyrin® in combination with staphylococcal infection, the second possibly by barbiturate (Pentymal), and the third by multiple drug provocation, sulphamethoxine (Bayrena®) probably being the decisive factor in the last resort. The salazopyrin patient survived but incurred a severe visual defect. The 2 of these cases with lethal course exhibited toxic injuries in the myocardium, liver and kidneys, in one case leading to uraemia, and both developed pulmonary oedema. The fourth case, also with fatal outcome, was not caused by a drug. It started as an ordinary mucocutaneous syndrome with eruption of erythema exsudativum multiforme type. The syndrome later developed into epidermolysis in connection with secondary staphylococcal infection.

Toxic epidermal necrolysis may possibly be classified under the febrile mucocutaneous syndrome, but it has such characteristic traits aetiologically, clinically, histopathologically and prognostically, that it should be accounted in independent form. Especially distinctive aetiologically is the provocative factor, as several agents have a significant influence in ordinary mucocutaneous syndromes but in epidermolysis they appear to be confined to staphylococci. The fourth case, however, illustrates how a mucocutaneous syndrome may change to epidermolysis through secondary infection with these bacteria.

The therapy in this ailment should be the same as for skin burns. Prophylactically, in exudative forms of mucocutaneous syndromes the aim should be to combat secondary staphylococcal infections.