A 24-year-old woman presents to the emergency department with a 3-day history of fever, malaise, and painful oral ulcers. On examination, she has extensive erythematous macules and papules on her trunk and extremities, with some lesions showing central blistering. Her oral mucosa, conjunctivae, and genital area are involved. She reports starting cotrimoxazole for a urinary tract infection 5 days ago. Temperature is 38.5°C, blood pressure 110/70 mmHg. Skin biopsy shows full-thickness epidermal necrosis with subepidermal blister formation. What is the most appropriate immediate management?

Explanation

## Diagnosis and Immediate Management **Key Point:** Stevens-Johnson Syndrome (SJS) is a severe cutaneous adverse drug reaction characterized by involvement of <10% body surface area (BSA), with mucosal involvement in >90% of cases. The clinical triad of fever, mucosal ulceration, and targetoid lesions with epidermal necrosis on histology confirms SJS. ### Immediate Management Priorities 1. **Drug Withdrawal** — The offending agent (cotrimoxazole in this case) must be stopped immediately. Continuation delays healing and increases mortality risk. 2. **Supportive Care** — This is the cornerstone of management: - Fluid and electrolyte replacement (large fluid losses from denuded skin) - Nutritional support (oral intake often impaired due to mucosal involvement) - Pain control - Infection prevention (barrier nursing, sterile dressings) 3. **Monitoring** — Assess for systemic complications (hepatic, renal, pulmonary involvement) ### Role of Systemic Corticosteroids **High-Yield:** The use of systemic corticosteroids in SJS/TEN remains controversial. Current evidence suggests: - Early high-dose corticosteroids (within 48 hours of onset) may reduce progression and mortality in severe cases - However, they do NOT accelerate healing and carry infection risk in an already immunocompromised state - Most guidelines recommend AGAINST routine corticosteroid use in established SJS unless there is evidence of systemic organ involvement - Supportive care alone is often sufficient for SJS (BSA <10%) ### Histopathology Correlation Full-thickness epidermal necrosis with subepidermal blister formation is pathognomonic for SJS/TEN and confirms the diagnosis. This distinguishes it from: - Erythema multiforme (superficial dermal inflammation, keratinocyte necrosis limited to basal layer) - Drug reaction with eosinophilia and systemic symptoms (DRESS) — perivascular infiltrate, no epidermal necrosis **Clinical Pearl:** The presence of mucosal involvement (oral, ocular, genital) in >90% of SJS cases makes this a systemic disease requiring intensive supportive care and monitoring for ocular sequelae (symblepharon, corneal scarring). ### Why Supportive Care is the Cornerstone SJS is fundamentally a disorder of keratinocyte apoptosis triggered by drug-immune interaction. Management focuses on: - Halting the trigger (drug withdrawal) - Supporting the patient through the acute phase (fluid, nutrition, infection prevention) - Preventing complications (ophthalmologic involvement, secondary infection, organ dysfunction) [cite:Harrison 21e Ch 56]