How will you manage a case of Stevens-Jhonson Syndrome in an adult?

I. Initial Assessment and Diagnosis

The initial assessment is paramount. A thorough history should be taken, focusing on medication use (including over-the-counter drugs and herbal remedies) in the weeks preceding symptom onset. Physical examination should document the extent of epidermal detachment, mucosal involvement (oral, ocular, genital), and systemic symptoms.

• Diagnostic Criteria: Diagnosis is primarily clinical, based on the characteristic appearance of target lesions, widespread epidermal detachment, and mucosal involvement.
• Differential Diagnosis: Rule out other conditions like scalded skin syndrome, bullous pemphigoid, and viral exanthems.
• Scoring Systems: SCORTEN (Severity-of-Illness Score for Toxic Epidermal Necrolysis) can be used to assess prognosis and guide management.

II. Acute Phase Management (First 72 Hours)

The acute phase focuses on resuscitation and preventing secondary complications.

A. Resuscitation and Stabilization

• Fluid Resuscitation: Aggressive intravenous fluid resuscitation is critical to counteract fluid loss from the damaged skin. Guided by urine output and hemodynamic parameters.
• Electrolyte Management: Monitor and correct electrolyte imbalances (sodium, potassium, calcium, magnesium).
• Pain Management: Adequate analgesia is essential. Opioids may be required, but careful monitoring for respiratory depression is necessary.
• Wound Care: Similar to burn management. Sterile dressings (e.g., silver sulfadiazine, biosynthetic dressings) should be applied to prevent infection. Avoid adherent dressings.
• Temperature Regulation: Maintain normothermia.

B. Supportive Care

• Nutritional Support: Early enteral nutrition is preferred. If not possible, parenteral nutrition should be considered.
• Respiratory Support: Monitor for airway compromise due to mucosal swelling. Intubation and mechanical ventilation may be required.
• Ocular Care: Frequent lubrication with artificial tears and ophthalmological consultation are crucial to prevent corneal damage and blindness.
• Infection Control: Strict infection control measures are essential. Prophylactic antibiotics are generally *not* recommended, but monitor closely for secondary infections (pneumonia, sepsis, wound infections).

C. Specific Therapies

• Causative Agent Withdrawal: Immediately discontinue all suspected medications.
• Intravenous Immunoglobulin (IVIG): May be considered, particularly early in the course, but evidence is mixed. Dosage varies (typically 1-2 g/kg over several days).
• Corticosteroids: Controversial. Some studies suggest benefit with early use, but others show increased risk of infection. Use should be individualized.
• Cyclosporine: Emerging evidence suggests potential benefit, particularly in early stages.

III. Post-Acute Phase and Recovery

Once the epidermal detachment stabilizes, the focus shifts to wound healing, preventing complications, and rehabilitation.

• Continued Wound Care: Continue sterile dressings and monitor for infection.
• Skin Grafting: Consider skin grafting for large areas of denuded skin.
• Physical Therapy: Prevent contractures and maintain joint mobility.
• Psychological Support: SJS/TEN can be emotionally traumatic. Provide psychological support and counseling.
• Long-Term Follow-up: Monitor for long-term sequelae, such as skin pigmentary changes, scarring, and ocular complications.

IV. Monitoring and Complications

Close monitoring is essential to detect and manage potential complications.

• Sepsis: Early recognition and aggressive treatment are crucial.
• Acute Respiratory Distress Syndrome (ARDS): Requires mechanical ventilation and supportive care.
• Multiple Organ Dysfunction Syndrome (MODS): Manage supportively.
• Ocular Complications: Dry eye, corneal ulcers, and blindness.
• Scarring and Contractures: Require physical therapy and potentially surgical intervention.