Epidermal Necrolysis: 60 Years of Errors and Advances

Y.K. Heng; H.Y. Lee; J.-C. Roujeau

Disclosures

The British Journal of Dermatology. 2015;173(5):1250-1254. 

In This Article

Causality

In 1990, Roujeau et al. contributed a review in which they wrote, 'Drugs are the most important, if not the only, cause of TEN. Infections are well-recognized causes of SJS but not of TEN.'[9] Both statements were erroneous. The hypothesis of different causes for SJS and TEN was incorrect, as was the suggestion that TEN had no cause other than drugs. The results of the SCAR case–control study indicated that the proportion of cases that were due to an associated medication ('etiologic fraction') was around 0·65. This figure was very similar for all groups of patients with EN, regardless of extent of involvement.[1] Further experience on larger numbers of cases confirmed that no more than 70–80% of cases are drug-induced, with similar percentages for SJS, TEN and SJS/TEN overlap.[10]

Drugs identified to be associated with high risk of causing EN include allopurinol, carbamazepine, phenytoin, lamotrigine, phenobarbital, cotrimoxazole and other anti-infective sulfonamides, sulfasalazine, oxicam nonsteroidal anti-inflammatory drugs and nevirapine.[11,12]

'Idiopathic' cases of EN are more common in children and only a very small percentage of these can be related reliably to infections such as Mycoplasma pneumoniae.[13] Gaining a better understanding of such 'idiopathic' cases remains a research priority.

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