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Stevens-Johnson syndrome/toxic epidermal necrolysis: initial assessment

BMJ 2024; 386 doi: https://doi.org/10.1136/bmj-2024-079986 (Published 13 September 2024) Cite this as: BMJ 2024;386:e079986
  1. Daniel Creamer, consultant dermatologist1,
  2. Tatiana Lumb, patient2,
  3. Carrie D Tibbles, emergency physician3,
  4. Haur Yueh Lee, consultant dermatologist4
  1. 1Department of Dermatology, King’s College Hospital, London, UK
  2. 2Patient author
  3. 3Department of Emergency Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA
  4. 4Department of Dermatology, Singapore General Hospital, Singapore
  1. Correspondence to D Creamer daniel.creamer{at}nhs.net

What you need to know

  • Consider a diagnosis of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) in a person with rash and multi-site mucositis who has been started on a new drug within the previous two months

  • Cutaneous pain is a prominent symptom in SJS/TEN and precedes blistering and sloughing of involved epidermis

  • Early transfer of patients to an intensive care unit or burns unit reduces mortality in SJS/TEN

A student in her late teens is started on lamotrigine for suspected epilepsy. Thirteen days later her eyes become red and itchy, and the following day she develops a sore throat, chapped lips, and widespread skin itching. She attends the emergency department, is diagnosed with a viral illness, and prescribed antihistamine. The next day she has a fever, malaise, facial rash, and oral ulceration. Within 24 hours the rash spreads to her torso and limbs and has started to blister. She is admitted to the intensive care unit of a specialist burns unit and over the next five weeks is treated for extensive skin loss with severe corneal and oral ulceration.

What is Stevens-Johnson syndrome/toxic epidermal necrolysis?

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) describes an acute, life threatening, mucocutaneous blistering disease resulting from epidermal and mucous membrane keratinocyte apoptosis.1 Blistering of less than 10% of body surface area (BSA) is known as Stevens-Johnson syndrome (SJS), and when greater than 30% BSA is referred to as toxic epidermal necrolysis (TEN). SJS-TEN overlap exists when BSA involvement lies between 10% and 30%.1 Estimated incidence is five or six cases per million per year.2 In 85% of patients with SJS/TEN, the disorder is caused by a hypersensitivity reaction to a drug. Around 15% of patients have no relevant medication exposure, and in these cases Mycoplasma pneumoniae or viral infections are often the cause, especially in children. During the active phase of SJS/TEN, loss of large …

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