What is the immediate management for a patient diagnosed with Stevens-Johnson syndrome?

Immediate Management of Stevens-Johnson Syndrome

The immediate management of Stevens-Johnson Syndrome (SJS) requires prompt identification and discontinuation of the culprit drug, immediate transfer to a burn center or intensive care unit, and implementation of supportive care measures to reduce mortality and morbidity. 1

Initial Assessment and Diagnosis

• Diagnostic Confirmation:

  • Perform a full physical examination, documenting:
    • Extent of skin involvement (% body surface area affected)
    • Presence of target lesions, purpuric macules, blisters, and epidermal detachment
    • Mucosal involvement (eyes, mouth, genitalia) 2
  • Obtain skin biopsy to confirm diagnosis and exclude other blistering disorders 2
  • Document the extent of erythema and epidermal detachment on a body map 2

• Risk Assessment:

  • Calculate SCORTEN within 24 hours of admission to predict mortality risk 1
  • Identify high-risk patients (those with drug triggers, underlying malignancy, or previous stem cell transplant) 2

Immediate Interventions

1. Drug Management:

   • Immediately discontinue any potential culprit drug 2, 1
   • Document all medications taken over the previous 2 months, including over-the-counter and complementary therapies 2
   • Record dates of drug initiation, dose changes, and discontinuation 2

2. Transfer to Specialized Care:

   • Transfer patients with >10% body surface area involvement to a burn center or specialized ICU without delay 1
   • Delayed transfer is associated with increased mortality 1

3. Environmental Control:

   • Place patient in a side room with controlled humidity and ambient temperature between 25-28°C 1
   • Use a pressure-relieving mattress to prevent pressure sores 1

4. Skin Management:

   • Handle skin carefully to minimize epidermal detachment 1
   • Leave detached epidermis in situ as a biological dressing 1
   • Apply non-adherent dressings to denuded dermis 1
   • Gently cleanse wounds using warmed sterile water, saline, or dilute chlorhexidine 1
   • Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis 1

5. Fluid Management:

   • Establish adequate intravenous fluid replacement through non-lesional skin 1
   • Note that fluid requirements are lower than predicted by burn formulas 1
   • Monitor fluid balance carefully 1

Specialized Care

1. Ophthalmological Management:

   • Arrange examination by an ophthalmologist within 24 hours of diagnosis 2, 1
   • Apply preservative-free lubricants every 2 hours 1
   • Perform daily ocular hygiene to remove inflammatory debris and break down conjunctival adhesions 1
   • Use topical antibiotics and corticosteroid drops under ophthalmologist supervision 1

2. Urogenital and Oral Management:

   • Examine urogenital tract during initial assessment 1
   • Apply white soft paraffin to urogenital skin/mucosae every 4 hours 1
   • Use silicone dressings on eroded areas to reduce pain and prevent adhesions 1
   • Clean mouth daily with warm saline mouthwashes 1
   • Use benzydamine hydrochloride rinse every 3 hours, particularly before eating 1

3. Pain Management:

   • Administer adequate background analgesia following WHO analgesic ladder principles 1
   • Use opiate-based regimens for moderate-to-severe pain 1
   • Consider patient-controlled analgesia (PCA) 1
   • Assess pain at least once daily using standardized tools 1

4. Infection Prevention:

   • Do not administer prophylactic systemic antibiotics 1
   • Treat only when clinical signs of infection are present 1
   • Monitor for sepsis, which may be masked by disease-associated fever 1
   • Obtain bacterial swabs from lesional skin for culture and sensitivity 2
   • Consider prophylactic anticoagulation with low molecular weight heparin for immobile patients 1

5. Laboratory Investigations:

   • Order complete blood count, C-reactive protein, renal and liver function tests, coagulation studies, glucose, electrolytes 2
   • Screen for infectious causes (mycoplasma, chlamydia, HSV, bacterial cultures) 2

Pharmacological Interventions

• Immunomodulatory Therapy:
  • Consider intravenous immunoglobulin (IVIG) in severe or steroid-unresponsive cases (total dose 3-4 g/kg) 1
  • Consider cyclosporine in severe or steroid-unresponsive cases 1
  • Systemic corticosteroids may be administered but may increase infection risk 1

Common Pitfalls to Avoid

1. Delayed recognition and transfer to specialized care
2. Failure to immediately discontinue the culprit drug
3. Inadequate fluid management and monitoring
4. Insufficient pain control
5. Prophylactic antibiotic use without signs of infection
6. Neglecting ophthalmological assessment and management
7. Underestimating the importance of wound care and skin handling

By following this comprehensive approach to the immediate management of SJS, clinicians can significantly improve patient outcomes and reduce the risk of long-term complications.