ABSTRACT
INTRODUCTION Adverse cutaneous drug reactions (ADRs) are frequent, affecting 2%–3% of all hospitalized patients. Fortunately, only about 2% of ADRs are severe and very few are fatal.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, severe, life-threatening diseases with a mortality rate reaching 30%, and only prompt recognition and diagnosis, along with referral to an intensive care unit or burn care unit might improve the prognosis and save the patient’s life.
Historical Background In his classic 1866 treatise “On Disease of the Skin,” Ferdinand von Hebra (1816-1880) precisely described and gave the name to erythema multiforme.1 In 1922, two American physicians, Albert Mason Stevens (1884-1945) and Frank Chambliss Johnson (1894-1934), described two patients, boys aged 7 and 8 years, who had “an extraordinary, generalized eruption with continued fever, inamed buccal mucosa, and severe purulent conjunctivitis”2 that was later given the name “Stevens-Johnson syndrome.” In 1950, Bernard A. Thomas divided EM into two categories: erythema multiforme minor (von Hebra) and erythema multiforme major, also known as Stevens-Johnson syndrome (SJS).3 In 1956, Alan Lyell (1917-2007) wrote the most highly cited contribution ever to appear in The British Journal of Dermatology: he described four patients with a scalding disease, which was later given the name toxic epidermal necrolysis (TEN), or the Lyell syndrome or Lyell disease.4,5 These severe, acute, life-threatening ADR were not classied and dened according to their clinical appearance and linked to their etiology and prognosis until around 1993.6
