Dermatology

Stevens-Johnson Syndrome

스티븐스-존슨 증후군

Drug-induced epidermal necrosis (<10% BSA)

How common
Rare but life-threatening
Typical age
All ages

What is it?

Drug-specific CD8+ T cells release granulysin causing widespread keratinocyte apoptosis.

Commonly affected: Mucosa (≥2 sites) + skin

How it develops

  1. Drug exposureSulfonamides, allopurinol, anticonvulsants
  2. T-cell activationHLA-B–restricted drug recognition
  3. Granulysin releaseInduces keratinocyte apoptosis
  4. Mucocutaneous sheddingWidespread mucocutaneous detachment

Symptoms

  • Mucosal involvementOral, ocular, genital ulcers
  • Positive NikolskyEpidermal detachment
  • Target lesionsAtypical targets
  • Fever/prodromeFever 1–3 days before rash

How it is examined

  • Drug historyNew drug 4–28 days prior
  • BSA detachment<10% SJS, >30% TEN
  • Ophthalmology consultRisk of vision loss

Imaging

Imaging not needed.

  • Clinical

For complications (e.g., pneumonia).

  • Pulmonary complications

Non-surgical care

  • Stop offending drugKey prognostic factor
  • Burn center admissionFluid, electrolyte, pain mgmt
  • Eye care, multidisciplinaryOphth, surgery, derm involved

Conservative options are generally tried first. Medications listed here can have side effects — discuss them with your prescriber.

When surgery is considered

None (supportive)

Procedures that may be discussed

  • IVIG, cyclosporine (controversial)

Outlook

SJS mortality 5%, TEN 25–50%. Predict with SCORTEN.

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