What is the management approach for a patient with delayed diagnosis of Stevens-Johnson syndrome?

Management of Delayed Diagnosis of Stevens-Johnson Syndrome

Patients with delayed diagnosis of Stevens-Johnson Syndrome (SJS) should be immediately transferred to a burn center or intensive care unit with experience in managing SJS, with implementation of multidisciplinary supportive care as the cornerstone of management. 1

Immediate Assessment and Triage

• Calculate SCORTEN score to predict mortality risk (score of 3 = 32% mortality risk) 1
• Document extent of erythema and epidermal detachment on a body map 1
• Perform skin biopsy to confirm diagnosis if not already done 1
• Identify and immediately discontinue any potential culprit drug 2, 1
• Assess for complications that may have developed due to delayed diagnosis:
  • Ocular involvement (corneal damage, symblepharon)
  • Respiratory complications
  • Secondary infections/sepsis
  • Urogenital complications

Supportive Care Priorities

Environment and Fluid Management

• Adjust ambient temperature to 25-28°C 1
• Place patient on a pressure-relieving mattress 1
• Provide adequate intravenous fluid replacement through non-lesional skin 1
• Monitor fluid balance carefully (requirements are lower than predicted by burn formulas) 1

Pain Management

• Provide adequate background analgesia following WHO analgesic ladder principles 1
• Consider opiate-based regimens for moderate-to-severe pain 1

Skin Care Approach

• Handle skin carefully to minimize shearing forces and epidermal detachment 1
• Apply bland emollients frequently to support barrier function 1
• Gently cleanse wounds using warmed sterile water, saline, or dilute chlorhexidine 1
• Leave detached epidermis in situ as a biological dressing 1
• Apply non-adherent dressings to denuded dermis 1
• Avoid aggressive debridement in the initial phase 1

Systemic Therapy Options

Corticosteroids

• For SJS with <10% BSA involvement: IV methylprednisolone 0.5-1 mg/kg 2
• For severe cases (≥10% BSA): IV methylprednisolone 1-2 mg/kg 2
• Convert to oral corticosteroids on response and taper over at least 4 weeks 2

Alternative Immunomodulatory Therapies

• Consider cyclosporine A (3 mg/kg daily for 7-10 days) in severe or steroid-unresponsive cases 2
• IVIG may be considered as an alternative in severe cases 2

Management of Specific Complications

Ocular Care

• Arrange urgent ophthalmological examination 1
• Apply preservative-free lubricants every 2 hours 1
• Consider topical antibiotics and corticosteroid drops under ophthalmologist supervision 1
• For severe cases, amniotic membrane transplantation may be needed to prevent long-term sequelae

Oral Care

• Clean mouth daily with warm saline mouthwashes 1
• Consider topical corticosteroids (betamethasone sodium phosphate 0.5 mg in 10 mL water as rinse-and-spit) 1
• Use benzydamine hydrochloride rinse every 3 hours, particularly before eating 1

Urogenital Care

• 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
• Consider catheterization to prevent urethral strictures 1

Infection Prevention and Management

• Monitor for signs of sepsis (may be masked by disease-associated fever) 1
• Do not administer prophylactic antibiotics without clinical signs of infection 1
• If infection suspected, obtain cultures and start broad-spectrum antibiotics 3
• Consider low molecular weight heparin as prophylactic anticoagulation for immobile patients 1

Follow-up Care

• Provide patients with written information about drugs to avoid 2
• Encourage patients to wear a MedicAlert bracelet 2
• Report the episode to pharmacovigilance authorities (e.g., MHRA's Yellow Card Scheme in the UK) 2
• Arrange follow-up with:
  • Dermatology within a few weeks of discharge 2
  • Ophthalmology (if eye involvement occurred) 2
  • Other specialists based on organ involvement 1

Monitoring for Long-term Sequelae

• Skin: scarring, pigmentation changes
• Eyes: dry eyes, symblepharon, corneal damage, vision loss
• Oral: xerostomia, dental caries
• Urogenital: strictures, synechiae
• Respiratory: bronchiolitis obliterans
• Psychological: post-traumatic stress disorder

Despite delayed diagnosis, aggressive supportive care and appropriate immunomodulatory therapy can still improve outcomes and reduce mortality in patients with SJS.