SJS/TEN

Stevens-Johnson syndrome and toxic epidermal necrolysis are delayed hypersensitivity emergencies defined by painful mucocutaneous injury and epidermal necrosis. Diagnosis is clinical, mucosal involvement is a major clue, and early priorities are stopping the trigger, assessing organ involvement, and arranging burn-center level care.

Recognition and diagnosis of SJS TEN

• Mucocutaneous diagnostic pattern: Painful erythematous or purpuric macules that blister and desquamate, plus mucosal erosions, should immediately raise concern for SJS/TEN; absence of mucosal involvement pushes you toward another diagnosis.
• Typical prodrome and spread: A viral-like prodrome of fever, sore throat, myalgias, and malaise often precedes the rash by about 3 days, with lesions starting on the face and thorax before spreading symmetrically.
• Pseudo Nikolsky finding: Pseudo-Nikolsky sign means epidermal shearing occurs over involved lesions only, a useful bedside distinction from pemphigus vulgaris or staphylococcal scalded skin syndrome.
• Trigger timing and causes: Medications are the classic inciting event, especially allopurinol, antiepileptics, and TMP-SMX, but Mycoplasma, HIV, HSV, and no identifiable trigger all remain on the table. We get into the timing nuances in the episode.
• Bedside workup priorities: The ED diagnosis is clinical, so labs and chest x-ray are for end-organ injury and complications rather than confirmation; skin, mouth, genitals, and eyes all need a deliberate exam.
• Ocular involvement frequency: Eye involvement is common, occurring in 60% to 100% of cases, so fluorescein examination matters early to catch corneal erosions, conjunctival ulceration, or pseudomembranes.

Management and prognostic assessment

• First critical intervention: Immediate withdrawal of the offending agent is the key disease-modifying step, while initial management follows ABCs with resuscitation and supportive care rather than empiric immunomodulators.
• Supportive care cornerstone: SJS/TEN behaves like a burn-level illness, with fluid resuscitation targeting euvolemia, electrolyte repletion, meticulous wound care, and multidisciplinary management at a burn center.
• Airway and pulmonary risk: Laryngeal, bronchial, or alveolar involvement can complicate these cases, and refractory hypoxia or airway compromise should lower your threshold for endotracheal intubation.
• Infection management approach: Sepsis is a leading cause of death, so broad-spectrum antibiotics are reserved for suspected superinfection such as cellulitis or pneumonia; prophylactic antibiotics are not recommended.
• Systemic therapy caution: Cyclosporine, infliximab, IVIG, steroids, and plasmapheresis are all discussed in practice, but the evidence is limited and burn-center input should come before starting them. We walk through that decision-making in the chapter.
• SCORTEN mortality signal: SCORTEN uses 7 bedside and laboratory variables to estimate mortality risk, and the danger rises sharply as points accumulate, especially in TEN rather than limited SJS.

References:

1. van Nispen C, Long B, Koyfman A. High risk and low prevalence diseases: Stevens Johnson syndrome and toxic epidermal necrolysis. Am J Emerg Med. 2024 Jul;81:16-22. PMID: 38631147.
2. Owen CE, Jones JM. Recognition and Management of Severe Cutaneous Adverse Drug Reactions (Including Drug Reaction with Eosinophilia and Systemic Symptoms, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis). Med Clin North Am. 2021 Jul;105(4):577-597. PMID: 34059239