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Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

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Drug Eruptions

Part of the book series: Updates in Clinical Dermatology ((UCD))

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Abstract

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are manifestations of a drug-induced mucocutaneous epithelial necrolysis syndrome. The two entities represent either end of a severity spectrum with SJS at the milder end (<10% body surface area epidermal detachment) and TEN at the severe end (>30% epidermal detachment). During the acute phase, SJS/TEN is associated with multiple morbidities and a high mortality (15–30%). There are a number of commonly implicated drugs including the antibacterial sulfonamides, allopurinol, aromatic anticonvulsants, nonsteroidal anti-inflammatory drugs of the oxicam family, and nevirapine. Granulysin appears to be the major cytotoxic molecule responsible for keratinocyte necrosis. Two or more mucous membranes are involved in almost all cases; initial skin lesions are purpuric macules and atypical targets. Ultimately SJS/TEN progresses to blistering and detachment of epidermal sheets. Patients with SJS/TEN should be admitted to a unit with specialist expertise in the management of skin loss syndromes and acute skin failure (specialized intensive care unit or burns unit). Survival is associated with an early diagnosis and supportive care directed at treating acute skin failure. There is no unambiguous evidence for significant benefit from any of the active interventions used in SJS/TEN (systemic corticosteroids, IVIg, ciclosporin). After the acute phase, survivors of SJS/TEN are commonly troubled by long-term mucocutaneous, ocular, and psychological sequelae.

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Acknowledgments

Prof. Nicolas de Prost, Prof. Pierre Wolkenstein, Mrs. Audrey Colin, patients’ association AMALYSTE.

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Authors and Affiliations

  1. Department of Dermatology and Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions Toxibul, APHP, Henri Mondor Hospital, Creteil, France

    Saskia Ingen-Housz-Oro

  2. Department of Dermatology, Henri Mondor Hospital, Creteil, France

    Tu-anh Duong & Olivier Chosidow

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  1. Saskia Ingen-Housz-Oro
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  2. Tu-anh Duong
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  3. Olivier Chosidow
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Correspondence to Olivier Chosidow .

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  1. Department of Dermatology and Allergy Centre, Singapore General Hospital, Singapore, Singapore

    Haur Yueh Lee

  2. Department of Dermatology, King’s College Hospital, London, UK

    Daniel Creamer

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Ingen-Housz-Oro, S., Duong, Ta., Chosidow, O. (2022). Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis. In: Lee, H.Y., Creamer, D. (eds) Drug Eruptions. Updates in Clinical Dermatology. Springer, Cham. https://doi.org/10.1007/978-3-031-09388-3_8

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