Stevens–Johnson Syndrome and TEN — Initial Management

Key Points

• Stop the suspected culprit drug immediately and avoid unnecessary new medications.
• Skin pain, mucosal erosions, and epidermal detachment in a recently medicated patient should trigger urgent SJS/TEN evaluation.
• Body surface area detachment distinguishes SJS, overlap, and TEN categories but does not replace severity assessment.
• Supportive care in an experienced burn or critical-care environment is the foundation of treatment.
• Early ophthalmology involvement is essential even when ocular symptoms initially appear mild.
• Do not give prophylactic systemic antibiotics routinely; treat proven or suspected infection based on clinical evidence.
• SCORTEN helps estimate mortality and should be interpreted as a prognostic tool.
• The evidence for systemic immunomodulatory therapies varies, so treatment should follow experienced specialist protocols.

Algorithm

1. Stop all suspected culprit drugs and obtain a precise medication start and stop timeline.
2. Assess airway, hemodynamics, temperature, pain, detached body surface area, and oral, ocular, genital, and respiratory mucosa.
3. Obtain CBC, electrolytes, renal and liver tests, glucose, bicarbonate, cultures when infection is suspected, and other testing for severity and alternatives.
4. Consult dermatology early and transfer significant disease to a burn center or ICU with SJS/TEN experience.
5. Provide wound care, fluids, analgesia, nutrition, thermoregulation, and thrombosis prevention as indicated.
6. Obtain urgent ophthalmology evaluation and additional mucosal specialists based on involved sites.
7. Calculate SCORTEN and reassess severity during the first days of hospitalization.
8. Use systemic immunomodulatory therapy only through a coordinated specialist and institutional protocol.

Clinical Synopsis & Reasoning

SJS and TEN lie on a spectrum defined by epidermal detachment: SJS involves less than 10% body surface area, TEN more than 30%, and overlap syndrome 10% to 30%. Patients often develop fever, malaise, sore throat, or eye symptoms followed by rapidly progressive tender erythematous or dusky skin, atypical targetoid lesions, blistering, epidermal sloughing, and mucosal erosions. Skin pain may precede obvious detachment and is an important clue.

Common triggers include sulfonamide antibiotics, certain aromatic anticonvulsants, allopurinol, oxicam NSAIDs, nevirapine, and other drugs, although infections and idiopathic cases occur. The highest-risk period is typically within the first several weeks after starting a culprit medication. A careful medication timeline is essential because stopping the responsible drug early is associated with better outcome. Differential diagnoses include erythema multiforme major, DRESS, acute generalized exanthematous pustulosis, pemphigus, staphylococcal scalded skin syndrome, burns, and autoimmune blistering disease.

Diagnosis is clinical and usually supported by skin biopsy when uncertainty exists. Evaluate the eyes, mouth, genital and urinary mucosa, and respiratory tract because mucosal injury can cause long-term disability. SCORTEN estimates mortality risk using age, malignancy, heart rate, detached or compromised surface area, serum urea, glucose, and bicarbonate. It is a prognostic tool rather than a substitute for clinical assessment and should be recalculated as disease evolves.

Treatment Strategy & Disposition

Immediately discontinue all nonessential suspected culprit medications. Transfer patients with significant detachment, hemodynamic instability, rapid progression, or major mucosal involvement to a center experienced in SJS/TEN, often a burn ICU or specialized critical-care unit. Supportive care includes thermoregulation, careful crystalloid replacement guided by hemodynamics and urine output, electrolyte management, analgesia, nutritional support, atraumatic wound care, and venous thromboembolism prophylaxis when appropriate.

Routine prophylactic systemic antibiotics are not recommended because they do not prevent sepsis and may add drug exposure; treat documented or strongly suspected infection promptly. Obtain early ophthalmology consultation because corneal and conjunctival injury can progress quickly and early ocular surface management affects long-term vision. Gynecology or urology should be involved for significant genital or urinary involvement, and airway specialists for respiratory mucosal disease.

Systemic immunomodulatory treatment remains an area of evolving evidence and institutional practice. Cyclosporine, corticosteroids, IVIG, and TNF-alpha inhibitors such as etanercept have been used, but the optimal regimen is not universally established. Early dermatology and burn-center protocols should guide these decisions. The emergency clinician's highest-certainty interventions are culprit withdrawal, supportive critical care, pain control, prevention of secondary injury, and rapid specialist coordination.

Epidemiology / Risk Factors

• SJS/TEN is rare but has high morbidity and mortality, especially as detached surface area increases.
• Most cases are drug-related, with risk concentrated in the first weeks after exposure to a high-risk medication.
• Older age, malignancy, large detached surface area, metabolic abnormalities, and tachycardia contribute to higher SCORTEN mortality estimates.
• Patients with HIV or selected genetic HLA backgrounds have increased risk with particular culprit drugs.
• Re-exposure to a causative drug can produce rapid and severe recurrence.

Investigations

SCORTEN Variables (Abbrev.)

Pharmacology

Prognosis / Complications

• Mortality rises substantially from SJS to extensive TEN and is influenced by age, comorbidity, sepsis, and organ dysfunction.
• Survivors can have chronic dry eye, corneal scarring, visual loss, oral and genital stenosis, dyspareunia, pigment change, nail abnormalities, and psychological trauma.
• Ocular outcomes are strongly influenced by severity and early specialist management.
• Drug avoidance usually prevents recurrence from the same culprit but accidental re-exposure can be catastrophic.
• Recovery can take weeks to months and often requires multidisciplinary follow-up.

Patient Education / Counseling

• Provide the patient with the exact generic and brand names of suspected culprit drugs and advise lifelong avoidance unless a specialist later proves otherwise.
• Encourage use of an allergy alert, medical identification, and communication of the reaction to every future prescriber and pharmacist.
• Explain that eye discomfort, photophobia, visual change, urinary difficulty, genital pain, or respiratory symptoms require specialist follow-up even after skin healing.
• Long-term dermatology and ophthalmology follow-up should be arranged, with gynecologic or urologic follow-up when genital mucosa was involved.
• Discuss emotional trauma, body-image concerns, sexual health, and post-intensive-care symptoms during recovery.
• Family members should understand that SJS/TEN itself is not contagious.

Notes

SCORTEN is useful for communication and prognostication but should not determine whether an obviously ill patient receives intensive supportive care. Repeat assessment is reasonable because variables can change during the first days.

Medication reconciliation is an emergency intervention in SJS/TEN. Continuing a culprit medication while awaiting biopsy can worsen injury, so a plausible high-risk drug should be stopped promptly unless there is a compelling reason not to.

References

1. American Burn Association Clinical Practice Guidelines — Link
2. American Academy of Dermatology SJS/TEN Resources — Link