What is the initial workup and management for a patient with Stevens-Johnson (Toxic Epidermal Necrolysis) syndrome?

Initial Workup and Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN)

The immediate management of SJS/TEN requires discontinuation of any potential culprit drug, prompt diagnostic workup, and transfer to a specialized unit (burn center or intensive care unit) for multidisciplinary supportive care. 1

Initial Assessment

History

• Document symptoms suggestive of SJS/TEN: prodromal illness (fever, malaise, upper respiratory symptoms), painful rash initially on face and chest, and involvement of mucosal sites 1
• Record when the rash first appeared and its progression 1
• Ask about respiratory symptoms (cough, dyspnoea, bronchial hypersecretion) 1
• Ask about gastrointestinal symptoms (diarrhea, abdominal distension) 1
• Determine the index date (onset of first symptom) 1
• Document all medications taken over the previous 2 months, including OTC and complementary therapies 1
• Create a timeline for each drug, noting start dates, dose changes, and discontinuation 1

Physical Examination

• Record vital signs, oxygen saturation, and baseline body weight 1
• Examine for target lesions, purpuric macules, blisters, and areas of epidermal detachment 1
• Examine all mucosal sites (eyes, mouth, genitalia) for mucositis, blisters, and erosions 1
• Document extent of erythema and epidermal detachment on a body map, estimating percentage of body surface area (BSA) involved 1
• Note that detachment should include both detached and detachable epidermis (Nikolsky positive areas) 1

Diagnostic Workup

Laboratory Tests

• Full blood count, ESR, C-reactive protein 1
• Urea and electrolytes, magnesium, phosphate, bicarbonate 1
• Glucose, liver function tests, coagulation studies 1
• Mycoplasma serology 1

Imaging

• Chest X-ray 1

Skin Biopsy

• Take a biopsy from lesional skin adjacent to a blister for histopathology 1
• Take a second biopsy from periblister lesional skin for direct immunofluorescence to exclude immunobullous disorders 1
• Histopathology typically shows epidermal necrosis, basal cell vacuolar degeneration, and subepidermal vesicle formation 1

Microbiology

• Swabs from lesional skin for bacteriology 1
• Consider additional testing for HSV, mycoplasma, or chlamydia if clinically indicated 1

Documentation

• Photograph skin lesions to document type and extent of involvement 1
• Calculate SCORTEN (severity-of-illness score for toxic epidermal necrolysis) to predict mortality risk 1

Initial Management

Immediate Actions

1. Discontinue any potential culprit drug causing SJS/TEN immediately 1
2. Establish peripheral venous access (through non-lesional skin if possible) 1
3. Initiate appropriate intravenous fluid resuscitation and maintain a fluid chart 1
4. Assess ability to maintain oral hydration and nutrition; insert nasogastric tube if needed 1
5. Insert urinary catheter if urogenital involvement causes dysuria/retention or to monitor output 1

Specialized Care

• Transfer to an intensive care unit or burn center under multidisciplinary care 2, 3
• Arrange ophthalmology consultation within 24 hours of diagnosis 1

Supportive Care

Skin Care

• Use silicone dressings (e.g., Mepitel) for eroded areas 1
• Consider modern dressings such as nanocrystalline that can remain in place longer 2

Mucosal Care

• Oral: Clean with warm saline mouthwashes daily, apply white soft paraffin to lips every 2 hours, use antiseptic oral rinse containing chlorhexidine twice daily, and consider potent topical corticosteroid mouthwash four times daily 1
• Urogenital: Apply white soft paraffin every 4 hours, use potent topical corticosteroid ointment once daily on non-eroded surfaces 1
• Eyes: Urgent ophthalmology consultation for appropriate eye care 1

Respiratory Support

• Monitor for respiratory symptoms and hypoxemia 1
• Early discussion with intensivist if respiratory compromise is present 1

Pharmacological Management

• The use of systemic corticosteroids remains controversial 4, 5
• If used, corticosteroids should only be administered during the progression phase and withdrawn as soon as possible 4
• Prednisone is FDA-approved for severe erythema multiforme (Stevens-Johnson syndrome) 6
• Consider cyclosporine as a potential treatment option under specialist supervision 1, 5
• Intravenous immunoglobulins have shown variable results 4, 5

Monitoring and Complications Prevention

• Daily assessment of vital signs and organ function 1
• Regular monitoring for infection, the most common cause of mortality 4
• Daily review of mucosal sites (oral, ocular, urogenital) 1
• Monitor for potential complications: septicemia, respiratory failure, renal dysfunction 7

Common Pitfalls and Caveats

• Delayed recognition and treatment significantly increases mortality 1
• Failure to immediately discontinue the culprit drug worsens prognosis 1
• Inadequate fluid resuscitation can lead to renal failure 1
• Neglecting eye care may result in permanent visual impairment 1
• Underestimating the extent of epidermal detachment can lead to inappropriate triage decisions 1
• Overlooking infectious causes, particularly mycoplasma in children and young adults 1

Discharge Planning

• Provide written information about drug(s) to avoid 1
• Encourage wearing a MedicAlert bracelet 1
• Document drug allergy in patient's notes and inform all healthcare providers 1
• Report the episode to pharmacovigilance authorities 1
• Arrange follow-up with dermatology and ophthalmology within weeks of discharge 1