**3. Steven Johnson syndrome/toxic epidermal necrolysis (SJS-TEN) overlap syndrome**

#### **3.1 History of SJS and TEN**

Steven Johnson Syndrome was first described in 1922 by American pediatrician named Albert Mason Stevens (1884–1945) and Frank Chambliss Johnson (1894–1934) who reported two cases of boys in New York City, United States, aged 7 years and 8 years presenting with an extraordinary generalized skin eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis [17]. Both these cases were misdiagnosed by primary care physicians as a case of "hemorrhagic measles" or "black measles". Similarly, the first description of Toxic Epidermal Necrolysis (TEN) also called Lyell syndrome was given by Scottish dermatologist Dr. Alan Lyell (1917–2007) in 1956 in four patients. Initially, these were considered as the toxic eruption that resembled severe burn or scalding of skin associated with erythematous plaques and widespread areas of epidermal detachment and was referred

**Figure 1.** *Epidermal involvement of the face and neck.*

**Figure 2.** *Epidermal involvement of limbs.*

## *Perspective Chapter: Drug-Induced Severe Cutaneous Adverse Reactions, Diagnostics... DOI: http://dx.doi.org/10.5772/intechopen.108651*

to as necrolysis due to excessive apoptotic keratinocytes [18]. Skin and mucous membranes were involved but with very little inflammation in the dermis referred to as the phenomenon of "dermal silence" by the dermatologist. This was an acute, rare, life-threatening mucocutaneous disease with an annual incidence of approximately 0.4–1.2 cases per million individuals with a mortality rate of more than 30% [19]. SCAR and EuroSCAR pooled data analysis was performed for children under 15 years of age that revealed that anti-bacterials class of drugs such as sulphonamides, antiepileptics such as phenobarbitol, lamotrigine, and carbamazepine was found to be strongly associated with SJS/TEN in this pediatric population [20]. Following is the case of toxic epidermal necrolysis involving the face and neck (**Figure 1**), trunk, and all four limbs (**Figure 2**) in an elderly female on cefixime and paracetamol treatment for dengue fever with no pre-existing comorbidities.
