What is the management approach for a patient diagnosed with Steven Johnson Syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer the patient without delay to a specialized burn unit or ICU with multidisciplinary expertise in managing extensive skin loss, as this is the single most critical intervention that directly impacts survival. 1, 2

Initial Assessment and Triage

• Calculate SCORTEN within the first 24 hours to predict mortality risk (0 parameters = 1% mortality; 5 parameters = 85% mortality) and guide intensity of care 1, 2
• Document all medications taken in the previous 2 months, including over-the-counter and herbal products, with exact start dates to identify the culprit drug 1, 2
• Perform full physical examination recording vital signs, oxygen saturation, baseline body weight, and calculate body surface area (BSA) involvement 1
• Order baseline investigations: complete blood count, electrolytes, liver and renal function, glucose, magnesium, phosphate, bicarbonate, coagulation studies, mycoplasma serology, and chest X-ray 1, 3
• Obtain skin biopsy from lesional skin adjacent to a blister for histopathology showing confluent epidermal necrosis with subepidermal vesicle formation 1, 2

Care Setting and Multidisciplinary Team

• Transfer patients with >10% BSA epidermal detachment to a burn center or ICU immediately, as delayed transfer significantly increases mortality 1, 2
• Barrier-nurse in a side room with ambient temperature raised to 25-28°C, on a pressure-relieving mattress with humidity control 1
• Convene a multidisciplinary team coordinated by dermatology and/or plastic surgery, including intensive care, ophthalmology, specialist skincare nursing, and additional specialists (respiratory, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, pharmacy) as needed 1, 3

Skin Management

Conservative Approach (All Patients)

• Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment 1, 3
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1, 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas 1, 2
• Leave detached epidermis in situ to act as a biological dressing; decompress blisters by piercing and expression or aspiration of fluid 1, 2
• Apply nonadherent dressings (Mepitel™ or Telfa™) to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2
• Consider silver-containing products/dressings for sloughy areas only, guided by local microbiological advice 1

Infection Prevention

• Do NOT use prophylactic systemic antibiotics, as this increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 3
• Take swabs for bacterial and candidal culture from three areas of lesional skin on alternate days throughout the acute phase 1, 3
• Administer targeted antimicrobial therapy only when clinical signs of infection are present (confusion, hypotension, reduced urine output, reduced oxygen saturation, or monoculture from multiple sites) 1, 2, 3

Fluid and Nutritional Management

• Establish adequate intravenous fluid replacement guided by urine output, avoiding overaggressive resuscitation that causes pulmonary, cutaneous, and intestinal edema 2, 3
• Insert urinary catheter if urogenital involvement causes significant dysuria/retention or to monitor output 1, 3
• Provide continuous enteral nutrition throughout the acute phase: 20-25 kcal/kg daily during catabolic phase, 25-30 kcal/kg during recovery 2, 4
• Insert nasogastric tube and institute nasogastric feeding if patient cannot maintain adequate oral nutrition 1, 2

Mucosal Management

Ophthalmologic Care

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

Oral Care

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

Urogenital Care

• Examine urogenital tract regularly during acute illness 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Pain Management

• Use validated pain assessment tools at least once daily 3
• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 1
• Administer intravenous opioid infusions for those not tolerating oral medication 3
• Consider patient-controlled analgesia where appropriate 3
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 3

Systemic Immunomodulatory Therapy

The evidence for systemic therapy remains debated, but early intervention within 72 hours shows the most promise. 1, 4

Corticosteroids

• Administer IV methylprednisolone 0.5-1 mg/kg daily (or equivalent) if started within 72 hours of onset, as FDA labeling specifically indicates methylprednisolone for severe erythema multiforme (Stevens-Johnson syndrome) 4, 5, 6
• Convert to oral corticosteroids on response, tapering over at least 4 weeks 4, 6
• Multiple studies suggest benefit when initiated early, particularly methylprednisolone pulse therapy 1, 2, 6

Cyclosporine

• Consider cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, as it has shown benefit in multiple studies with reduced mortality compared to predicted SCORTEN rates 1, 2, 4, 6
• May be used alone or in combination with corticosteroids 1, 6

Additional Considerations

• Intravenous immunoglobulin (IVIg) has been studied but evidence remains mixed and inconclusive 1, 7, 6
• The guideline development group notes insufficient evidence to demonstrate clear benefit or harm from these interventions, ideally practiced under specialist supervision in the context of clinical study or case registry 1

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation against venous thromboembolism in immobile patients 4
• Provide proton pump inhibitor to protect against upper gastrointestinal stress ulceration if enteral nutrition cannot be established 4

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid permanently, including all potentially cross-reactive medications 1, 2, 4
• Encourage wearing a MedicAlert bracelet or amulet bearing the name of the culprit drug 2, 4
• Document drug allergy in patient's medical records and inform all healthcare providers involved in their care 2, 4
• Report the adverse drug reaction to pharmacovigilance authorities (MHRA in U.K.) 1, 2, 4
• Send explanatory letter to GP outlining the disorder, culprit medication, potential complications, and follow-up plan 1
• Arrange dermatology follow-up within weeks of discharge 2, 4
• Schedule ophthalmology follow-up to monitor for chronic ocular complications that may develop months after acute disease 1, 2, 4
• Inform patients about expected fatigue and lethargy for several weeks following discharge, requiring convalescence period 1, 2
• Consider referral to support groups such as SJS Awareness U.K. 1, 2
• Refer to local social services for needs assessment when appropriate 1

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality - early transfer reduces mortality while delays adversely affect outcomes 2, 3, 4, 7, 8
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 1, 2, 3, 4
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 2, 3
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 2, 4
• Continued use of the culprit medication worsens the condition and increases mortality 2
• Neglecting to monitor for sepsis, which is the most common cause of death in SJS/TEN 3, 7, 8

Special Considerations for Pediatric Patients

• Infection causes up to 50% of pediatric SJS/TEN cases - test for infective triggers and consult infectious disease team in all pediatric cases 2
• Manage in age-appropriate specialist units with pediatric intensivists and skin loss specialists 2
• High-risk children need quicker transfer to specialized care 2