Approach

SJS and TEN are similar to second-degree burns in terms of physiologic effects.

Treatment requires a multidisciplinary team so that patients receive optimal daily wound care, nutrition, critical care, pain management, and supportive care.[75] Transfer to a burn center, a specialized wound care center, or a dermatology intensive care unit is recommended for patients with SJS/TEN.[63][75]

Exact treatment will depend on the extent of skin involvement, but the same general principles are applicable to both SJS and TEN. Treatment should be initiated on a case-by-case basis depending on individual patient presentation.[76][77]

Both conditions have a tendency to progress over the course of several days, so patients should be monitored closely.

Immediate care

Upon diagnosis, the causative agent should be identified and withdrawn immediately.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

Drugs most frequently implicated in SJS/TEN are listed in the etiology section. See Etiology.

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.

[Figure caption and citation for the preceding image starts]: Rule of ninesFrom Dr Sheridan's personal collection [Citation ends].com.bmj.content.model.Caption@70fe7147

[Figure caption and citation for the preceding image starts]: Lund-Browder diagramFrom Dr Sheridan's personal collection [Citation ends].com.bmj.content.model.Caption@56b53826

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:[75]

  • Initial body surface area (BSA) 70% or more

  • Progression of BSA involved from day of hospitalization (DOH) 1 to DOH 3 of ≥15%

  • Underlying neurologic 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] 

Provided the patient does not require therapeutic anticoagulation on admission, immobile patients should receive prophylactic low molecular weight heparin.[75] 

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.[75]

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][75]

Potential oral, ocular, and urogenital involvement should be evaluated as part of the initial assessment of all patients with SJS/TEN.[75] 

Wound care

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

Conservative management (antishear strategy) recommends:[75] 

  • Preserving the detached epidermis as a biologic dressing

  • Limiting dressing changes

  • Using an air-fluidized bed

  • Using nonadherent dressings

  • Using lysis and drainage of wounds only for patient comfort

  • Cleansing wounds with sterile water, normal saline, 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-epithelialization

  • Considering use of nonadherent, silver-impregnated primary dressings for antibacterial properties, reduced requirement for dressing changes, and improved patient comfort

  • Using secondary absorptive dressings to control exudate.

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 biologic dressing, such as xenograft (pigskin), allograft (cadaver skin), or synthetic dressing.[78] With healing, consider a nonadherent silver-impregnated dressing for dressing changes and improved patient comfort.

UK guidance recommends that patients should be transferred to burn unit for surgical debridement and wound care if they have TEN (>30% BSA epidermal loss) and evidence of the following:[63]

  • Clinical deterioration

  • Extension of epidermal detachment

  • Subepidermal pus

  • Local sepsis

  • Wound conversion and/or delayed healing.

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

Fluid management

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][75]

If the patient can take fluids orally, encourage them to do so. Otherwise, start intravenous fluids such as lactated Ringer solution or 0.9% sodium chloride to hydrate the patient. One case series study indicates that approximately 2 mL/kg/% TBSA may be adequate if there are no other complications.[79]

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][75]

Increase or decrease fluid resuscitation based on the urine output.

Management of pain

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][75] Patients will require more pain medication during dressing changes.[63][75]

If mild pain is not controlled with acetaminophen, an oral opioid such as tramadol should be considered.[75] Morphine or fentanyl may be necessary for patients with moderate to severe pain scores. Low-dose ketamine can be considered as an alternative or adjuvant therapy for SJS/TEN pain.[75] Gabapentin and pregabalin both address neuropathic pain and may decrease opioid consumption in both the acute and healing phases for patients with SJS.[75] 

Nonsteroidal anti-inflammatory drugs should be avoided due to the increased risk of renal or gastric injury.[75]

Intravenous immune globulin and cyclosporine

There are no clear indications regarding administration of intravenous immune globulin (IVIG). Some clinicians give IVIG to patients with a rapidly progressing rash involving at least 6% TBSA. Others only give IVIG when 20% TBSA is affected. There are no definitive randomized controlled trials to guide treatment. The current literature consists of small retrospective and prospective series. Reviews of these small clinical trials have shown some benefit and no major complications with the use of IVIG.[2][24][42][44][80][81][82] For SJS/TEN overlap and TEN patients, many burn centers give IVIG when there is rapid progression of the rash, and when the patient is clinically deemed a candidate.

There have been sporadic case reports of successful treatment of TEN with cyclosporine. In a retrospective chart review of 71 patients with SJS/TEN, cyclosporine was associated with fewer deaths than expected (standardized mortality ratio 0.43) while IVIG was associated with excess mortality (standardized mortality ratio 1.43).[83] Another study of 16 patients treated with cyclosporine demonstrated a lower mortality rate than predicted by the SCORTEN score.[84] Results from one small (n=29) phase 2 open-label trial suggest that cyclosporine 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 TBSA of 77% treated with cyclosporine and plasmapheresis.[85] Other meta-analyses suggest that cyclosporine use in epidermal necrolysis is effective in reducing the risk of death.[76][86][87][88]

Oral involvement

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 exam on presentation and on a daily basis during the acute phase of the illness.[63][75]

Applying petrolatum ointment on the lips every 2 hours throughout the acute illness is recommended.[63][75]

Pain management

To provide short-term pain relief, oral rinses or sprays with local anesthetic, anti-inflammatory, and analgesic properties (e.g., viscous lidocaine, benzydamine) should be given, particularly before eating or oral cleansing.[63][75] 

Topical oral coating agents are recommended for pain reduction in patients with oral mucosal involvement.[75] 

Oral hygiene

The patient’s mouth should be cleaned daily with warm saline mouthwashes or an oral sponge.[63][75] 

To reduce bacterial colonization of the mucosa an antiseptic oral rinse should be used (e.g., diluted chlorhexidine).[63][75] 

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][75] 

Nutrition

Nutritional support should be administered orally if possible. For patients unable to tolerate oral intake, provide enteral feeding through a nasogastric tube.[75] Nasogastric tube placement should be avoided in patients with nasopharyngeal mucosa involvement.

Nutritional support of 30-35 kcal/kg should be delivered, maintaining close glycemic control.[75] If the patient's caloric intake is not sufficient with enteral nutrition, it should be supplemented via the parenteral route.[75] 

Ocular involvement

All patients manifesting signs and symptoms of SJS/TEN should have an ophthalmologic consultation and a full exam upon admission, and on a daily basis during the acute phase of illness, until it is established that there is no visual deterioration.[63][75][89] Follow-up should be determined on a case-by-case basis.[75] 

Ocular lubricants (drops and ointments) should be administered every 2 hours during the acute stage of illness for patients with ocular involvement.[63][75] 

Daily exam of the entire ocular surface (eyelid skin, eyelid margin, conjunctiva, and cornea) is recommended.[75] 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.[75]

Resting eyelid position should be assessed for lagophthalmos, especially in unconscious patients, as prevention of corneal exposure is essential.[63][75] 

Amniotic membrane transplantation should be considered during the initial evaluation of any patient thought to have SJS/TEN, and at each follow-up exam during the acute phase.[75] 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.[89]

Urogenital involvement

Urogenital involvement occurs in approximately 70% of women and men with SJS/TEN.[90][91] This can result in erosions of the scrotum/labia, penis/vulva; dysuria; hematuria; urinary retention; and long-term sequelae such as urethral stenosis and scarring, xerosis, phimosis, dyspareunia, chronic pain, bleeding, sexual dysfunction, infertility, and anxiety.[75] 

Exam of the urogenital tract of all patients with SJS/TEN is recommended upon initial assessment and daily during hospitalization.[63][75] 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, reduce adhesion formation, and facilitate healing during the acute phase of the disease.[75] For women with suspected vaginal involvement, an intravaginal dilator can be used to apply either:[63][75]

  • A nonsteroidal 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.

Tapering of the topical corticosteroid 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.[75]

Medication can be alternated with estrogen cream to encourage healing of the vaginal mucosa.[75]

Intravaginal dilators can be in place for a maximum of 24 hours before replacement.[75] 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.[75] 

Occupational and physical therapy

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

Use of this content is subject to our disclaimer