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Chap. 15 - SEVERE, ACUTE ADVERSE CUTANEOUS DRUG REACTIONS I: STEVENS–JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS

Published online by Cambridge University Press:  07 September 2011

By
Ronni Wolf  and
Batya B. Davidovici
Edited by
Ronni Wolf ,
Batya B. Davidovici ,
Jennifer L. Parish  and
Lawrence Charles Parish
Ronni Wolf
Affiliation:
Hebrew University–Hadassah Medical School
Batya B. Davidovici
Affiliation:
Kaplan Medical Center
Ronni Wolf
Affiliation:
Kaplan Medical Center, Rehovot, Israel
Batya B. Davidovici
Affiliation:
Kaplan Medical Center, Rehovot, Israel
Jennifer L. Parish
Affiliation:
Jefferson Medical College of Thomas Jefferson University
Lawrence Charles Parish
Affiliation:
Jefferson Medical College of Thomas Jefferson University
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Summary

ADVERSE CUTANEOUS drug reactions (ADRs) are frequent, affecting 2%–3% of all hospitalized patients. Fortunately, only approximately 2% of ADRs are severe, and few are fatal.

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, severe, life-threatening diseases with a mortality rate reaching 30%. Only prompt recognition, diagnosis, and 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 precisely described and gave the name to erythema multiforme (EM). In 1922, two American physicians, Stevens and Johnson, described two patients, boys 7 and 8 years old, who had “an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis” that was later given the name “Stevens–Johnson syndrome.” In 1950, Thomas divided EM into two categories: erythema multiforme minor (von Hebra) and erythema multiforme major, also known as SJS. In 1956, Alan Lyell wrote the most highly cited article 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. These severe, acute, life-threatening ADRs were not classified and defined according to their clinical appearance and/or linked to their etiology and prognosis until around 1993.

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Information
Emergency Dermatology , pp. 154 - 161
Publisher: Cambridge University Press
Print publication year: 2011

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