Epidermal Necrolysis: 60 Years of Errors and Advances

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


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

In This Article

Attempts to Clarify the Confusion Around Erythema Multiforme Major, Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

The concept of the EM/SJS/TEN spectrum was challenged by the observation that early lesions in SJS and TEN often have a different appearance from those in EM and that herpes virus infection was recognized to be a trigger for EM but not TEN.[7]

In an effort to resolve the confusion, an international group of investigators reviewed several hundred photographs of historical cases and proposed a classification based on the extent of blisters/erosions as well as the individual pattern and topographical distribution of the skin lesions.[8] Table 2 shows the classification of EMM and SJS/TEN that was proposed then and remains widely used.

A large study consisting of 552 patients and 1720 controls was performed by the Severe Cutaneous Adverse Reactions (SCAR) study group using the disease classification in Table 2. Results of this study showed that EMM differed from SJS and TEN in terms of demographics, associated diseases, causes and severity.[1] These differences, shown in Table 3, suggested that EMM is a disease entity separate from SJS and TEN, which in turn were shown to exist as a continuum differing only in the extent of skin involvement. EMM can be distinguished from SJS and TEN on the basis of simple clinical criteria (pattern and distribution of individual cutaneous lesions).[1]