What is the treatment approach for a patient diagnosed with Stevens-Johnson Syndrome (SJS)?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer the patient to a specialized burn unit or ICU with multidisciplinary expertise, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care as the cornerstone of management. 1, 2

Immediate Actions and Triage

• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk and guide intensity of care 1, 2, 3
• Transfer patients with >10% body surface area epidermal detachment to a burn center or ICU without delay, as early transfer reduces mortality 1, 2, 3
• Obtain skin biopsy from lesional skin adjacent to a blister for histopathology showing confluent epidermal necrosis with subepidermal vesicle formation 2, 3
• Document all medications taken in the previous 2 months, including over-the-counter and herbal products, with exact start dates 2, 3

Supportive Care Framework

Fluid Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that can lead to pulmonary, cutaneous, and intestinal edema 2, 4, 3
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 2, 4
• Consider using the formula: body weight/% BSA epidermal detachment to determine replacement volumes 3

Wound Care

• Handle skin gently to minimize shearing forces and prevent further epidermal detachment 2, 4, 3
• Leave detached epidermis in situ to act as a biological dressing 1, 2
• Apply bland emollients (50% white soft paraffin with 50% liquid paraffin) frequently over the entire epidermis, including denuded areas 2, 4, 3
• Use nonadherent dressings (such as Mepitel or Telfa) to denuded dermis with secondary foam or burn dressings to collect exudate 4, 3
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 4, 3

Pain Management

• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 4
• Use intravenous opioid infusions for those not tolerating oral medication 3
• Consider patient-controlled analgesia where appropriate, and sedation or general anesthesia for dressing changes 3

Infection Prevention

• Do not administer prophylactic systemic antibiotics as this increases skin colonization with resistant organisms, particularly Candida albicans 1, 4, 3
• Monitor for clinical signs of infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 3
• Institute targeted antimicrobial therapy only when clinical signs of infection are present 1, 4, 3
• Obtain regular skin swabs for bacterial and candidal culture from lesional skin 4, 3

Mucosal Management

Ocular Care

• Arrange ophthalmology consultation within 24 hours of diagnosis with daily examinations throughout the acute phase 1, 2, 4, 3
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 4
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 4
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 2, 4
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 2, 4
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 4

Oral Care

• Examine the mouth as part of initial assessment with daily oral review during acute illness 4
• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 4
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 2, 4
• Apply antiseptic oral rinse twice daily to reduce bacterial colonization 2, 4
• Use topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 2
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 4

Urogenital Care

• Catheterize all patients to help prevent urethral strictures and monitor urine output 2, 3
• Perform regular examination of urogenital tract during acute illness 2, 4
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 2
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Systemic Immunomodulatory Therapy

Ciclosporin (cyclosporine) at 3 mg/kg daily for 10 days, tapered over 1 month, has shown benefit with reduced mortality compared to predicted rates in multiple studies. 1, 2, 4

• Systemic corticosteroids, particularly IV methylprednisolone pulse therapy (1000 mg IV), may be beneficial if started within 72 hours of onset 1, 2, 4
• The British Association of Dermatologists notes that evidence for IVIg, systemic corticosteroids, or ciclosporin is not definitive, and ideally such interventions should be practiced under specialist supervision 1

Nutritional Support

• Deliver continuous enteral nutrition throughout the acute phase, providing 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery 4
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 4

Environmental Considerations

• Place patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 2, 3
• Use barrier nursing techniques in a side room controlled for humidity 3

Multidisciplinary Team Requirements

• Coordinate care through a multidisciplinary team including dermatology, intensive care, burn surgery, ophthalmology, and specialist skincare nursing 1, 2, 3
• Include additional specialists based on organ involvement: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy 3

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 1, 2, 4
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 2, 4
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 1, 2, 4
• Report the adverse drug reaction to pharmacovigilance authorities (MHRA Yellow Card Scheme in the U.K.) 1
• Arrange ophthalmology follow-up within a few weeks of discharge for all patients with eye involvement during the acute phase 1
• Arrange dermatology or burn plastic surgery follow-up within a few weeks of discharge 1
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 1, 4
• Consider referral to support groups such as SJS Awareness U.K. 1, 4
• Monitor for chronic complications including ocular damage (corneal scarring, chronic dry eye, symblepharon), cutaneous sequelae (pigmentation changes, scarring), and urogenital sequelae (urethral strictures, vaginal synechiae) 1, 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk 2, 3
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 1, 4, 3
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 2, 4, 3
• Failure to involve ophthalmology early leads to permanent visual impairment 2, 3
• Continued use of the culprit medication worsens the condition and increases mortality 2
• Neglecting psychological evaluation and support during recovery 1