Advanced Supportive Care & Complication Prevention in SJS/TEN
Learning Objective
Recommend supportive care and monitoring to manage complications of Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) and their treatments.
1. Respiratory Support
Mucosal sloughing of the tracheobronchial tree is a severe complication that can lead to airway obstruction and Acute Respiratory Distress Syndrome (ARDS). Proactive airway management is critical to prevent life-threatening events.
Key Interventions
- Early Recognition of Airway Compromise: Be vigilant for stridor, hoarseness, dysphagia, drooling, a rising PaCO₂, or refractory hypoxemia despite supplemental oxygen.
- Secure the Airway Electively: Coordinate with anesthesia or ENT specialists to secure the airway before severe mucosal edema and friability make intubation hazardous. Have difficult-airway equipment readily available.
- Indications for Mechanical Ventilation: Consider intubation for PaO₂/FiO₂ ratio <200, evidence of airway compromise, altered mental status, or refractory hypoxemia.
- Lung-Protective Ventilation (ARDS Protocol):
- Tidal volume: 6 mL/kg of predicted body weight
- Plateau pressure: ≤30 cm H₂O
- Use moderate Positive End-Expiratory Pressure (PEEP)
- Permissive hypercapnia is generally well-tolerated
- Adjunctive Therapies: For severe ARDS (PaO₂/FiO₂ <150), consider prone positioning. Frequent therapeutic bronchoscopy is essential for removing sloughed mucosal debris, assessing airway patency, and obtaining samples for culture.
Clinical Pearls
Plan for the Worst: Schedule elective intubation in a controlled setting like an operating room or ICU before severe mucosal edema develops. A reactive, emergent intubation is far more dangerous.
Protect the Lungs: Strict adherence to lung-protective ventilation settings significantly reduces the risk of ventilator-induced lung injury (barotrauma) and improves outcomes in SJS/TEN-associated ARDS.
2. Hemodynamic Management
Patients with SJS/TEN experience massive fluid losses through denuded skin, leading to hypovolemic shock similar to burn patients. The goal is to balance fluid resuscitation and vasopressor support to maintain end-organ perfusion while minimizing tissue edema.
Key Interventions
- Fluid Resuscitation: Lactated Ringer’s is the preferred crystalloid. Titrate fluids to achieve a Mean Arterial Pressure (MAP) ≥65 mm Hg and a urine output of 0.5–1 mL/kg/h.
- Invasive Monitoring: An arterial line is essential for continuous blood pressure monitoring. Use central venous pressure or dynamic indices (e.g., stroke volume variation) to guide fluid responsiveness. Mixed venous O₂ saturation can help assess the adequacy of oxygen delivery.
- Vasopressor Therapy:
- First-line: Norepinephrine (0.01–0.05 μg/kg/min), titrated to MAP ≥65 mm Hg.
- Adjuncts: Consider vasopressin (0.03 units/min) as a catecholamine-sparing agent. Use epinephrine (0.01–0.1 μg/kg/min) if there is evidence of myocardial depression.
- Echocardiography for Shock: If cardiogenic shock is suspected (e.g., ejection fraction <40%), add dobutamine (2–10 μg/kg/min). Be cautious with fluid administration in patients with left ventricular dysfunction.
Clinical Pearls
Norepinephrine First: Early initiation of norepinephrine can restore vascular tone, improve microcirculatory perfusion, and limit the total volume of resuscitation fluids, thereby reducing edema.
Dynamic Over Static: Use dynamic indices of preload (like stroke volume variation) over static ones (like CVP) to more accurately predict which patients will benefit from additional fluids.
3. ICU-Related Complication Prophylaxis
Immobilized, critically ill SJS/TEN patients are at high risk for preventable complications. A proactive, bundled approach is essential.
| Complication | Recommended Prophylaxis | Key Considerations |
|---|---|---|
| Venous Thromboembolism (VTE) | Enoxaparin 40 mg SC daily or Heparin 5,000 units SC q8h. | Use mechanical devices (SCDs) if anticoagulation is contraindicated. Dose-adjust enoxaparin for renal impairment (30 mg SC daily if CrCl <30 mL/min). |
| Stress Ulcers | Pantoprazole 40 mg IV daily. | Reassess the need daily. Discontinue prophylaxis once major risk factors (e.g., mechanical ventilation) are resolved to reduce infection risk. |
| Line-Associated Infections | Maximal sterile barrier insertion, chlorhexidine skin prep, daily line necessity audit. | Strict adherence to bundles is key. Remove unnecessary lines promptly. Change peripheral IVs every 72-96 hours. |
| Sepsis | Avoid prophylactic antibiotics. Obtain cultures before starting empiric therapy. | Obtain blood, urine, respiratory, and wound cultures for any new fever or sign of sepsis. De-escalate broad-spectrum antibiotics based on culture results. |
Clinical Pearls
Combined VTE Prophylaxis: In high-risk, immobilized patients, combining pharmacologic and mechanical VTE prophylaxis may offer the best protection against deep vein thrombosis and pulmonary embolism.
Deprescribe Acid Suppression: Discontinue proton pump inhibitors as soon as enteral feeding is established and other major risk factors resolve. This helps reduce the risk of hospital-acquired pneumonia and C. difficile infection.
4. Management of Iatrogenic Complications
Therapies used to manage SJS/TEN, such as corticosteroids and cyclosporine, can cause significant adverse effects that require vigilant monitoring and management.
Key Interventions
- Corticosteroid-Induced Hyperglycemia: Check blood glucose every 4 hours. Initiate an insulin infusion for levels persistently >180 mg/dL, targeting a range of 140–180 mg/dL. Transition to a basal-bolus subcutaneous insulin regimen when the patient is stable.
- Cyclosporine Nephrotoxicity: Establish a baseline creatinine and monitor it daily. If serum creatinine rises >30% over baseline, reduce the cyclosporine dose by ~25%. Target trough levels of 150–300 ng/mL and avoid concurrent use of other nephrotoxic agents (e.g., NSAIDs, vancomycin).
- Electrolyte Management: Check sodium, potassium, and magnesium every 12–24 hours. Maintain potassium >4 mEq/L and magnesium >2 mg/dL to prevent arrhythmias. Correct free water deficits slowly to avoid dangerous osmotic shifts in the brain.
Clinical Pearls
Moderate Glucose Control: The target glucose range of 140–180 mg/dL for most critically ill patients provides the best balance between mitigating the risks of hyperglycemia (e.g., infection, poor wound healing) and avoiding the dangers of iatrogenic hypoglycemia.
Protect the Kidneys: When using calcineurin inhibitors like cyclosporine, vigilant renal function monitoring is not optional—it is an essential component of the therapy to prevent irreversible kidney damage.
5. Wound Care & Infection Surveillance
The denuded skin is a major source of fluid loss, pain, and infection. Meticulous wound care is a cornerstone of SJS/TEN management, aiming to preserve skin integrity, minimize pain, and detect infection early.
Key Interventions
- Dressings: Use non-adherent materials such as silicone-based or petroleum-impregnated gauze. Change dressings every 24–48 hours under sterile conditions to minimize pain and contamination.
- Biologic Barriers: If available, consider using amniotic membrane grafts on denuded areas. These can reduce inflammation, alleviate pain, and promote re-epithelialization.
- Daily Inspection: Systematically inspect the entire skin surface daily for new blisters, expanding erythema, or signs of purulence. If systemic signs of infection (fever, leukocytosis) develop, obtain cultures from wounds, blood, urine, and respiratory secretions.
- Isolation Precautions: Implement strict contact precautions. Use dedicated equipment for the patient and enforce rigorous hand hygiene for all staff and visitors.
Clinical Pearls
Preserve the Blister Roof: Whenever possible, leave the roof of a blister intact. It serves as a natural, sterile biologic dressing that protects the underlying dermis.
Culture Before Antibiotics: Resist the urge to start antibiotics for fever alone. In SJS/TEN, fever is often due to the underlying inflammatory process. Defer antibiotic therapy until cultures are obtained and interpreted to avoid unnecessary drug exposure and resistance.
6. Nutritional & Metabolic Support
SJS/TEN induces a hypermetabolic, hypercatabolic state similar to severe burns. Meeting these increased demands is crucial for wound healing and immune function.
Key Interventions
- Caloric and Protein Goals: Target 25–30 kcal/kg/day and a high protein intake of 1.5–2 g/kg/day to support healing.
- Enteral Nutrition: Initiate feeding within 24 hours if the patient is hemodynamically stable. If significant oropharyngeal involvement exists, a post-pyloric feeding tube is preferred to bypass the damaged mucosa. Consider high-protein, immune-modulating formulas.
- Thermoregulation: Maintain the ambient ICU room temperature at 30–32 °C (86–90 °F). Use warming blankets or other devices to prevent hypothermia, which consumes calories and impairs coagulation.
- Metabolic Monitoring: Assess nutritional status weekly with markers like prealbumin or nitrogen balance studies, adjusting the feeding regimen as needed.
Clinical Pearls
Early Enteral Nutrition is Key: “If the gut works, use it.” Early feeding supports gut mucosal integrity, reduces bacterial translocation, and is associated with a lower risk of infection.
Prevent Hypothermia: Significant heat loss occurs through denuded skin. Preventing hypothermia is vital to conserve calories, reduce metabolic stress from shivering, and maintain normal enzymatic function.
7. Multidisciplinary Goals of Care
SJS/TEN carries a high mortality rate. Structured, patient-centered discussions are essential to align the intensity of medical therapy with the prognosis and the patient’s values.
Key Interventions
- Ethical Framework: Discussions should address four key domains: medical indications, patient preferences, quality of life, and contextual factors.
- Prognostication with SCORTEN: Calculate the SCORTEN score within the first 24 hours and consider repeating it on day 3 to estimate mortality risk. A score ≥3 predicts a mortality rate of over 35%.
- Palliative Care Involvement: Engage the palliative care team early for expert management of refractory symptoms like pain and dyspnea, as well as for providing psychosocial support to the patient and family.
- Communication: Schedule regular family meetings. Use clear, jargon-free language. Document advance directives and revisit care goals as the patient’s clinical status changes.
Clinical Pearls
Early and Often: Goals-of-care meetings should be initiated early, not just when care is perceived as futile. This proactive approach improves alignment between the medical team and the family and can reduce decisional conflict.
Prognosis is Dynamic: A care plan is not set in stone. It is crucial to reassess and adjust the plan as the clinical picture evolves, ensuring that the care provided remains consistent with the patient’s goals and prognosis.
References
- Seminario-Vidal L, Kroshinsky D, Malachowski SJ, et al. Society of Dermatology Hospitalists supportive care guidelines for the management of SJS/TEN in adults. J Am Acad Dermatol. 2020;82(3):609–619.e4.
- Finfer S, Chittock DR, Su SY, et al. Intensive vs conventional glucose control in critically ill patients. N Engl J Med. 2009;360(13):1283–1297.
- González-Herrada C, Rodríguez-Martín S, Cachafeiro L, et al. Cyclosporine use in epidermal necrolysis is associated with an important mortality reduction. J Invest Dermatol. 2017;137(10):2092–2100.
- Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 2000;115(2):149–153.
- Creamer D, Walsh SA, Dziewulski P, et al. U.K. guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults. Br J Dermatol. 2016;174(6):1194–1227.
