Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

all patients

1st line – urgent evaluation + withdrawal of causative agent

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1st line – 

urgent evaluation + withdrawal of causative agent

Upon diagnosis, the causative agent should be identified and withdrawn immediately. Usually it will be a new medication prescribed for the patient within the past 2-3 weeks prior to onset of the rash.

Drugs most frequently implicated in SJS/TEN are listed in the aetiology section. See Aetiology.

Patients with SJS/TEN should be assessed in the same way as a patient with cutaneous burns, using a structured approach to evaluate airway, breathing, and circulation. See Cutaneous burns (Diagnosis approach).

Arterial blood gases and oxygen saturation will help determine patient clinical respiratory status.

An immediate assessment of total body surface area (TBSA) involvement is required to assess severity of disease. Various methods can be used, such as the Wallace rule of 9s, Palmer, the Lund-Browder burn estimate chart, or SCORTEN.[55][57] Wallace rule of 9s Opens in new window See Diagnosis approach.

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The greater the percentage of TBSA involved, the greater the fluid requirement.[56]

Consider intubation and early tracheostomy in patients with oral involvement and one of the following: initial body surface area (BSA) 70% or more; progression of BSA involved from day of hospitalisation (DOH) 1 to DOH 3 of ≥15%; underlying neurological diagnosis prevents airway protection; documented airway involvement on direct laryngoscopy.

Evidence suggests that for patients who are intubated, mortality rates increase to over 50%.[54]

Potential oral, ocular, and urogenital involvement should be evaluated as part of the initial assessment of all patients with SJS/TEN.[74]

Plus – venous thromboembolism prophylaxis

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Plus – 

venous thromboembolism prophylaxis

Treatment recommended for ALL patients in selected patient group

Provided the patient does not require therapeutic anticoagulation on admission, immobile patients should receive prophylactic low molecular weight heparin (e.g., enoxaparin).[74] 

Mechanical thromboprophylaxis with graduated compression stockings or intermittent pneumatic compression is recommended for acutely ill patients at increased risk of thrombosis who are bleeding or at high risk for major bleeding.[74]

Primary options

enoxaparin: children and adults: consult specialist for guidance on dose

Plus – conservative or surgical wound care

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Plus – 

conservative or surgical wound care

Treatment recommended for ALL patients in selected patient group

Wound care can follow either a conservative or surgical approach to debridement and wound care depending on the needs of the individual patient.[63]  

Conservative management (anti-shear strategy) recommends: preserving the detached epidermis as a biological dressing; limiting dressing changes; using an air-fluidised bed and non-adherent dressings; using lysis and drainage of wounds only for patient comfort; cleansing wounds with sterile water or dilute chlorhexidine when dressings are changed; applying an emollient to the whole epidermis to enhance skin barrier function, reduce fluid loss, and encourage re-epithelialisation; considering use of a non-adherent silver-impregnated primary dressing for antibacterial properties, reduced requirement for dressing changes, and improved patient comfort; using secondary absorptive dressings to control exudate.[74]

The surgical approach for wound care for SJS/TEN patients generally follows practice in burn management: once the surgical debridement of detached epidermis is achieved, wounds are covered with a biological dressing, such as xenograft (pigskin), allograft (cadaver skin), or synthetic dressing.[77] With healing, consider a non-adherent silver-impregnated dressing for dressing changes and improved patient comfort. 

UK guidance recommends that patients should be transferred to a burn unit for surgical debridement and wound care if they have TEN (>30% body surface area epidermal loss) and evidence of the following: clinical deterioration, extension of epidermal detachment, sub-epidermal pus, local sepsis, wound conversion, and/or delayed healing.[63]

Once the skin has regenerated (after about 2-3 weeks), emollients can be useful to keep it supple and prevent drying out.

Plus – analgesia

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analgesia

Treatment recommended for ALL patients in selected patient group

Analgesia should be given based on the severity of symptoms to ensure comfort at rest. Pain level should be evaluated once every 4 hours and assessed at least once daily using a validated pain tool.[63][74] Patients will require more pain medication during dressing changes.[63][74]

If mild pain is not controlled with paracetamol, an oral opioid such as tramadol should be considered.[74] Morphine or fentanyl may be necessary for patients with moderate to severe pain scores. Low-dose ketamine an be considered as an alternative or adjuvant therapy for SJS/TEN pain.[74] Gabapentin and pregabalin both address neuropathic pain and may decrease opioid consumption in both the acute and healing phases for SJS/TEN patients.[74] 

Non-steroidal anti-inflammatory drugs should be avoided due to the increased risk of renal or gastric injury.[74] 

Primary options

paracetamol: children: consult specialist for guidance on dose; adults (oral): 500-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day; adults <51 kg body weight (intravenous): 15 mg/kg intravenously every 4-6 hours when required, maximum 60 mg/kg/day; adults ≥51 kg body weight (intravenous): 1000 mg intravenously every 4-6 hours when required, maximum 4000 mg/day (3000 mg/day if risk factors for hepatotoxicity)

Secondary options

tramadol: adults: 50-100 mg orally (immediate-release) every 4-6 hours when required, maximum 400 mg/day

Tertiary options

morphine sulfate: children: consult specialist for guidance on dose; adults: 2.5 to 10 mg intravenously/intramuscularly/subcutaneously every 2-6 hours when required, or 0.8 to 10 mg/hour intravenous infusion, or 10-30 mg orally (immediate-release) every 4 hours when required, adjust dose according to response

OR

fentanyl: children and adults: consult specialist for guidance on dose

OR

ketamine: children and adults: consult specialist for guidance on dose

OR

gabapentin: children and adults: consult specialist for guidance on dose

OR

pregabalin: children and adults: consult specialist for guidance on dose

Plus – fluid and electrolyte management

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Plus – 

fluid and electrolyte management

Treatment recommended for ALL patients in selected patient group

If a patient is vomiting frequently, dehydration may occur. In addition, depending on the extent of the skin sloughing, the patient may be losing significant amounts of fluids through the denuded skin surface. Electrolyte and fluid balance should be monitored daily.[63][74]

Patients who can take fluids orally should be encouraged to do so. Otherwise, start intravenous fluids such as lactated Ringer's solution or 0.9% sodium chloride to hydrate the patient. One case series study indicated that approximately 2 mL/kg/% total body surface area may be adequate if there are no other complications.[78]

Regardless of the calculated quantity of fluid required, the clinical evaluation of patient response is extremely important. Fluid resuscitation is monitored by urine output. It is important that an adult has a urine output of 0.5 mL/kg/hour (30-50 mL/hour) and children weighing <30 kg have an output of 1 mL/kg/hour.[63][74] Increase or decrease fluid resuscitation based on the urine output.

Plus – nutritional support

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nutritional support

Treatment recommended for ALL patients in selected patient group

Oral involvement occurs in the majority of patients with SJS/TEN, resulting in impaired oral intake.[63][74] 

Nutritional support should be administered orally if possible. For patients unable to tolerate oral intake, provide enteral feeding through a nasogastric tube.[74] Nasogastric tube placement should be avoided in patients with nasopharyngeal mucosa involvement.

Nutritional support of 30-35 kcal/kg should be provided, maintaining close glycaemic control.[74] If the patient’s caloric intake is not sufficient with enteral nutrition, it should be supplemented via the parenteral route.[74] 

Consider – proton-pump inhibitor

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Consider – 

proton-pump inhibitor

Additional treatment recommended for SOME patients in selected patient group

Patients at high risk of bleeding, and those in whom enteral nutrition cannot be established, should be given a proton-pump inhibitor to prevent stress-related gastritis and intestinal ulceration.[63][74] 

Treatment is usually for up to 14 days; however, patients may require treatment for longer depending on the size of the burn.

Primary options

omeprazole/sodium bicarbonate: children: consult specialist for guidance on dose; adults: 40 mg orally initially, followed by 40 mg in 6-8 hours on day 1, then 40 mg once daily

More

Consider – oral supportive care

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Consider – 

oral supportive care

Additional treatment recommended for SOME patients in selected patient group

Oral involvement occurs in the majority of patients with SJS/TEN, resulting in pain, impaired oral intake, and poor oral hygiene. Patients should have an oral examination on presentation and on a daily basis during the acute phase of the illness.[63][74] 

Applying petrolatum ointment on the lips every 2 hours throughout the acute illness is recommended.[63][74] 

To provide short-term pain relief, oral rinses or sprays with local anaesthetic, anti-inflammatory, and analgesic properties (e.g., viscous lidocaine, benzydamine) should be given, particularly before eating or oral cleansing.[63][74] 

Topical oral coating agents are recommended for pain reduction in patients with oral mucosal involvement.[74] 

The patient’s mouth should be cleaned daily with warm saline mouthwashes or an oral sponge.[63][74] 

To reduce bacterial colonisation of the mucosa, an antiseptic oral rinse should be used (e.g., diluted chlorhexidine).[63][74] 

Either a potent topical corticosteroid mouthwash (e.g., dexamethasone) or an ultrapotent topical corticosteroid ointment is recommended during the acute phase of the illness.[63][74] 

Consider – ocular supportive care

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Consider – 

ocular supportive care

Additional treatment recommended for SOME patients in selected patient group

All patients manifesting signs and symptoms of SJS/TEN should have an ophthalmological consultation and a full examination upon admission. To preserve vision and reduce complications, ophthalmological follow-up should continue on a daily basis during the acute phase of illness, until it is established that there is no visual deterioration.[63][74][88] Follow-up should be determined on a case-by-case basis.[74] 

Ocular lubricants (drops and ointments) should be administered every 2 hours during the acute stage of illness for patients with ocular involvement.[63][74]

Daily examination of the entire ocular surface (eyelid skin, eyelid margin, conjunctiva, and cornea) is recommended.[74] This should include assessing for forniceal and tarsal conjunctival epithelial defects and early symblephara by everting the eyelids with eyes rotated.

All patients should have fluorescein staining to detect corneal injuries, small foreign objects or particles in the eye, and abnormal tear production.[74]

Resting eyelid position should be assessed for lagophthalmos (incomplete or abnormal closure of the eyelid), especially in unconscious patients, as prevention of corneal exposure is essential.[63][74] 

Amniotic membrane transplantation should be considered during the initial evaluation of any patient thought to have SJS/TEN, and at each follow-up examination during the acute phase.[74] Coverage of the entire ocular surface with amniotic membrane, together with intensive short-term topical corticosteroids, during the acute phase of SJS and TEN has been shown to be associated with preservation of good visual acuity and an intact ocular surface.[88]

Consider – urogenital supportive care

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Consider – 

urogenital supportive care

Additional treatment recommended for SOME patients in selected patient group

Urogenital involvement occurs in approximately 70% of women and men with SJS/TEN.[89][90] This can result in erosions of the scrotum/labia, penis/vulva; dysuria; haematuria; urinary retention; and long-term sequelae such as urethral stenosis and scarring, xerosis, phimosis, dyspareunia, chronic pain, bleeding, sexual dysfunction, infertility, and anxiety.[74] 

Examination of the urogenital tract of all patients with SJS/TEN is recommended upon initial assessment and daily during hospitalisation.[63][74]  A urinary catheter should be inserted if urogenital involvement is causing significant dysuria/retention.[63]

The vulvar/urogenital skin/mucosa should be coated with an ointment and/or ointment gauze to help reduce pain and adhesion formation, and facilitate healing, during the acute phase of the disease.[74]

For women with suspected vaginal involvement, an intravaginal dilator can be used to apply a non-steroidal ointment (e.g., petrolatum jelly), with reapplication as frequently as necessary to maintain barrier protection and/or a high-potency corticosteroid ointment if active inflammation is observed.[63][74] Tapering of the topical corticosteroid use should be based on clinical improvement.  

If vaginal candidiasis is suspected, obtain a potassium hydroxide preparation and fungal culture and initiate treatment with a suitable antifungal medication.[74]

Medication can be alternated with oestrogen cream to encourage healing of the vaginal mucosa.[74]

Intravaginal dilators can be in place for a maximum of 24 hours before replacement.[74] If patients are not comfortable using an intravaginal dilator, medication can be applied with a vaginal applicator. 

Menstrual suppression may reduce the risk of vaginal adenosis and endometriosis, and can be considered in women with severe genital mucosal involvement.[74]

Consider – intravenous immunoglobulin

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Consider – 

intravenous immunoglobulin

Additional treatment recommended for SOME patients in selected patient group

There are no clear indications regards administration of intravenous immunoglobulin (IVIG); this treatment may be used by some clinicians depending on individual patient presentations.

Some clinicians give IVIG to patients with a rapidly progressing rash involving ≥6% of total body surface area (TBSA). Others only give IVIG when 20% TBSA is affected. There are no definitive randomised controlled trials to guide treatment.

IVIG is reserved for patients who have a rapid progression of their mucocutaneous signs and symptoms; comorbidities also determine the type of treatment that the patient will receive.

For SJS/TEN overlap and TEN patients, many burn centres give IVIG when there is rapid progression of the rash, and when the patient is clinically deemed a candidate.

Particular caution is required in patients with renal impairment.[2][81]

Doses used vary widely and are based on literature case reports, anecdotes, and small series.[44][79][80][81]

Primary options

normal immunoglobulin human: children and adults: consult specialist for guidance on dose

Consider – ciclosporin

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all patients
Consider – 

ciclosporin

Additional treatment recommended for SOME patients in selected patient group

There are no definitive randomised controlled trials to guide treatment. In a retrospective chart review of 71 patients with SJS/TEN, ciclosporin was associated with fewer deaths than expected (standardised mortality ratio 0.43) while intravenous immunoglobulin was associated with excess mortality (standardised mortality ratio 1.43).[82] Another study of 16 patients treated with ciclosporin demonstrated a lower mortality rate than predicted by the SCORTEN score.[83] Results from one small (n=29) phase 2 open-label trial suggest that ciclosporin can reduce mortality and the progression of epidermal detachment among patients with SJS/TEN. There was one death in a small study of 12 patients with an average total body surface area of 77% treated with ciclosporin and plasmapheresis.[84] Other meta-analyses suggest that ciclosporin use in epidermal necrolysis is effective in reducing the risk of death.[75][85][86][87]

Primary options

ciclosporin: children and adults: consult specialist for guidance on dose

Consider – physiotherapy and occupational therapy

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all patients
Consider – 

physiotherapy and occupational therapy

Additional treatment recommended for SOME patients in selected patient group

Patients can develop limitations in mobility with decreased strength. Arrange for daily exercises within the patient's capability with a physiotherapist and, if needed, an occupational therapist.[44]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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