Model Answer
0 min readIntroduction
Stevens-Johnson Syndrome (SJS) and its more severe form, Toxic Epidermal Necrolysis (TEN), are rare, life-threatening mucocutaneous reactions typically triggered by medications or infections. Characterized by widespread epidermal detachment, SJS/TEN requires prompt diagnosis and aggressive management to reduce morbidity and mortality. Early identification of the offending agent and supportive care are crucial. The distinction between SJS and TEN is based on the percentage of body surface area (BSA) affected – SJS involves <10% BSA detachment, TEN >30%, and overlap SJS/TEN involves 10-30%.
Pointwise Management of Stevens-Johnson Syndrome (SJS)
The management of SJS is multifaceted and requires a multidisciplinary approach. The following points outline the key aspects of care:
1. Initial Assessment & Stabilization
- Diagnosis: Clinical diagnosis based on characteristic skin lesions and mucosal involvement. Biopsy can confirm diagnosis but should not delay treatment.
- Causative Agent Identification: Thorough medication history and investigation for potential infectious triggers. Immediate discontinuation of suspected drugs.
- Severity Assessment: Utilize scoring systems like SCORTEN (Score for Toxic Epidermal Necrolysis) to assess prognosis and guide management.
- Airway Management: Assess for airway compromise due to mucosal involvement. Early intubation may be necessary.
- Fluid Resuscitation: Aggressive intravenous fluid resuscitation to address fluid loss from skin damage. Monitor urine output closely.
2. Supportive Care
- Wound Care: Similar to burn management. Sterile dressings, debridement of necrotic skin (avoid aggressive debridement). Silver sulfadiazine or biosynthetic dressings can be used.
- Pain Management: Adequate analgesia is crucial. Opioids may be required.
- Nutritional Support: Early enteral nutrition is preferred. Parenteral nutrition may be necessary if enteral route is not feasible.
- Infection Control: Strict infection control measures. Prophylactic antibiotics are generally *not* recommended, but treat any documented infections aggressively.
- Ophthalmological Care: Frequent ophthalmological examinations to prevent corneal damage. Lubricating eye drops and topical steroids may be used.
- Oral Hygiene: Meticulous oral hygiene to prevent secondary infections.
3. Specific Therapies (Controversial & Evolving)
- Intravenous Immunoglobulin (IVIG): May be beneficial in early stages, particularly in children. Evidence is mixed.
- Corticosteroids: Use is controversial. Some studies suggest benefit if administered early, while others show increased risk of infection.
- Cyclosporine: Emerging evidence suggests potential benefit in reducing mortality.
- Plasma Exchange: May remove causative agents or inflammatory mediators, but evidence is limited.
4. Monitoring
- Vital Signs: Continuous monitoring of vital signs, including heart rate, blood pressure, and respiratory rate.
- Fluid Balance: Strict monitoring of fluid intake and output.
- Electrolytes: Frequent monitoring of electrolytes.
- Skin & Mucosal Involvement: Daily assessment of BSA detachment and mucosal involvement.
- Signs of Infection: Vigilant monitoring for signs of secondary infection.
- Organ Function: Monitor renal and hepatic function.
5. Long-Term Follow-up
- Skin Pigmentation Changes: Patients may experience post-inflammatory hyperpigmentation.
- Scarring: Potential for scarring, requiring dermatological management.
- Ocular Sequelae: Long-term ophthalmological follow-up to monitor for chronic ocular complications.
Conclusion
Effective management of SJS requires a rapid, multidisciplinary approach focused on identifying and eliminating the causative agent, providing aggressive supportive care, and considering adjunctive therapies based on individual patient factors and evolving evidence. Early recognition, prompt intervention, and meticulous monitoring are crucial for improving patient outcomes and minimizing long-term sequelae. Ongoing research is needed to refine treatment strategies and improve our understanding of this life-threatening condition.
Answer Length
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