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.

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.

3. ICU-Related Complication Prophylaxis

Immobilized, critically ill SJS/TEN patients are at high risk for preventable complications. A proactive, bundled approach is essential.

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.

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.

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.

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.