What is the management and treatment approach for a patient suspected of having Steven Johnson syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer the patient without delay to a specialized burn center or ICU with multidisciplinary expertise in managing SJS/TEN, particularly when body surface area involvement exceeds 10%. 1

Initial Assessment and Triage

• Calculate SCORTEN within the first 24 hours to predict mortality risk, with scores ranging from 0-7 corresponding to mortality rates from 1% to 99%. 1, 2 This severity assessment guides the intensity of care required.

• Document the exact date of rash onset and record all medications taken in the previous 2 months, including over-the-counter and herbal products, with their start dates. 2, 3 Most symptoms begin 4-28 days after drug initiation. 4

• Obtain skin biopsy from lesional skin adjacent to a blister for histopathology showing confluent epidermal necrosis with subepidermal vesicle formation. 2, 3 A second biopsy from periblister skin for direct immunofluorescence excludes immunobullous disorders. 3

• Perform baseline laboratory testing including complete blood count, C-reactive protein, urea and electrolytes, magnesium, phosphate, glucose, liver function tests, and coagulation studies. 3

Transfer and Care Setting

Patients with >10% body surface area (BSA) epidermal loss must be admitted without delay to a burn center or ICU with experience treating SJS/TEN. 1 Three studies and a systematic review demonstrate that rapid admission to a burn center is associated with improved survival, while delayed transfer increases mortality. 1

• Barrier-nurse in a side room controlled for humidity on a pressure-relieving mattress with ambient temperature raised to 25-28°C. 1, 2

• Establish multidisciplinary team coordination led by a specialist in skin failure (dermatology or plastic surgery), including intensive care, ophthalmology, and specialist skincare nursing. 1, 2 Additional input may be required from respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy. 1

Supportive Care

Fluid Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding overaggressive replacement that leads to pulmonary, cutaneous, and intestinal edema. 2, 3 The cutaneous defect in SJS/TEN is analogous to a superficial burn. 1

• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes. 2, 3 Consider urinary catheterization when clinically indicated. 3

Skin and Wound Management

• Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment. 1 Day-to-day bedside care should be undertaken by specialist nurses familiar with skin fragility disorders. 1

• Leave detached epidermis in situ to act as a biological dressing. 1, 2 Decompress blisters by piercing and expression or aspiration of fluid. 2

• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000). 2, 3

• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every few hours. 1, 2, 3 Frequent application supports barrier function, reduces transcutaneous water loss, and encourages re-epithelialization. 1

• Use nonadherent dressings (such as Mepitel or Telfa) on denuded dermis with secondary foam or burn dressings to collect exudate. 1, 2, 3 Appropriate dressings reduce fluid and protein loss, limit microbial colonization, control pain, and accelerate re-epithelialization. 1

• Consider surgical debridement with biosynthetic xenograft or allograft following failure of conservative management, characterized by clinical deterioration, extension of epidermal detachment, local sepsis, delayed healing, or wound conversion. 1

Infection Prevention and Management

Do not use prophylactic systemic antibiotics as indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans. 1, 2, 3 This is a critical pitfall to avoid. 2

• Monitor carefully for signs of infection including confusion, hypotension, reduced urine output, reduced oxygen saturation, and increased skin pain. 1, 3 Fever from SJS/TEN itself complicates detection of secondary sepsis. 1, 3

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days. 2, 3

• Institute targeted antimicrobial therapy only when clinical signs of infection appear. 1, 2, 3 Systemic sepsis is the most frequent cause of death in SJS/TEN. 1

• Monitor C-reactive protein and neutrophilia as indicators of sepsis. 1, 2

Nutrition 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. 2

• Consider nasogastric feeding when oral intake is precluded by buccal mucositis. 2

Pain Management

• Use validated pain assessment tools at least once daily. 2, 3

• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain. 2

• Consider patient-controlled analgesia where appropriate, or sedation/general anesthesia for patient handling, repositioning, and dressing changes. 3

Mucosal Management

Ophthalmologic Care

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness. 2, 3 Neglecting eye care may result in permanent visual impairment. 3

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness. 2, 3

• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions. 2

• Use topical antibiotics when corneal fluorescein staining or ulceration is present. 2

• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage. 2

• Consider amniotic membrane transplantation in the acute phase, which demonstrates significantly better visual outcomes compared to medical management alone. 2

Oral Care

• Perform daily oral review during the acute illness. 2

• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness to reduce risk of fibrotic scars. 2

• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating. 2

• Use antiseptic oral rinse twice daily to reduce bacterial colonization. 2

• Apply 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. 2

Urogenital Care

• Perform regular examination of the urogenital tract during acute illness. 2

• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours. 2

• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation. 2

• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output. 2

Systemic Immunomodulatory Therapy

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 rates. 2, 3

Consider systemic corticosteroids, particularly early methylprednisolone pulse therapy, if started within 72 hours of onset. 2, 3 Prednisone is FDA-approved for severe erythema multiforme (Stevens-Johnson syndrome). 5

• Avoid thalidomide (an anti-TNF agent) as it was associated with excess deaths in one randomized trial. 2

• IVIg has equivocal evidence—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75). 2

Respiratory Management

• Urgent discussion with an intensivist is required when respiratory symptoms and hypoxemia are present on admission, with rapid transfer to ICU or burn center. 2

• Perform fiberoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium. 2

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients. 2

• Provide proton pump inhibitor if enteral nutrition cannot be established. 2

• Consider recombinant human G-CSF for neutropenic patients. 2

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications. 2

• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug. 2

• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care. 2

• Report the adverse drug reaction to national pharmacovigilance authorities. 2

• Arrange follow-up with dermatology and ophthalmology within weeks of discharge. 3

• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence. 2

• Consider referral to support groups such as SJS Awareness U.K. 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk. 1, 3

• Continued use of the culprit medication will worsen the condition and increase mortality. 3

• Indiscriminate prophylactic antibiotics increase skin colonization with resistant organisms. 1, 2, 3

• Overaggressive fluid resuscitation can lead to pulmonary, cutaneous, and intestinal edema. 2, 3

• Failure to involve ophthalmology early can lead to permanent visual sequelae. 2, 3

• Overlooking infectious causes, particularly mycoplasma in children and young adults, can worsen prognosis. 3