The British Association of Dermatologists has updated their guidelines for the diagnosis and management of the full spectrum of Stevens-Johnson Syndrome (SJS), Toxic Epidermal Necrolysis (TEN), and SJS-TEN overlap in the acute phase of disease in adults. It provides up-to-date, evidence-based recommendations for the diagnosis and management of the full spectrum of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and SJS–TEN overlap in adults during the acute phase of the disease.
SUMMARY OF RECOMMENDATIONS
1. Initial Assessment on Presentation:
- (i) Ask about symptoms suggestive of SJS/TEN, including a prodromal illness (fever, malaise, upper respiratory tract symptoms); onset of a painful rash, initially on the face and chest; and involvement of mucosal sites (eyes, mouth, nose, genitalia).
- Note the date when the rash first appeared and document progression of the eruption.
- Ask about symptoms indicating the involvement of the respiratory tract: a cough, dyspnoea, bronchial hypersecretion and haemoptysis.
- Ask about symptoms indicating bowel involvement: diarrhoea and abdominal distension.
- Determine the index date (date of onset of the adverse reaction) by asking when the patient developed the first symptom or sign of the disorder, for example sore throat, rash, skin pain and sore eyes/mouth.
- Record previous or ongoing medical problems; ask specifically about a history of recurrent HSV infections and chest infections.
- Record all medicines taken over the previous2 months, including over-the-counter (OTC) and complementary/alternative therapies; document the date.
- Perform a full physical examination, including baseline body weight, and record the vital signs, including oxygen saturation
- Order a set of investigations: FBC, U&E, LFT, glucose, magnesium, phosphate, bicarbonate, mycoplasma serology, CXR, skin biopsy
- Initiate a primary management plan including establishing peripheral venous access and in case patient cannot maintain adequate nutrition orally, insert a nasogastric tube and institute nasogastric feeding.
- Insert a urinary catheter if urogenital involvement is causing significant dysuria/retention
2. Determination of Drug Causality:
Identify causative agent and withdraw immediately.
3. Prognostic scoring:
Calculate SCORTEN within first 24 hours.
4. Care setting:
- An MDT should be convened, coordinated by a specialist in skin failure, usually dermatology and/or plastic surgery, and including clinicians from intensive care, ophthalmology and skincare nursing
- Patients with > 10% BSA epidermal loss should be admitted without delay to a burn centre or ICU with experience of treating patients with SJS/TEN and facilities to manage the logistics of extensive skin loss wound care
- Patients must be barrier-nursed in a side room controlled for humidity, on a pressure-relieving mattress with the ambient temperature raised to between 25 °C and 28 °C
5. Skin Management Regimen 1 (Applicable to all patients in all settings):
- Employ strict barrier nursing to reduce nosocomial infections
- Take swabs for bacterial and candidal culture from three areas of lesional skin, particularly sloughy or crusted areas, on alternate days throughout the acute phase
- Administer systemic antibiotics only if there are clinical signs of infection
6. Skin Management Regimen 2 (this may involve a conservative and/or surgical approach based on the specialist MDT’s daily review of the individual needs of the patient):
Conservative approach in all patients as follows:
- Regular cleansing of wounds and intact skin by irrigating gently using warmed sterile water, saline or an Antimicrobial agent like chlorhexidine (1/5000)
- Apply a greasy emollient, 50% white soft paraffin with 50% liquid paraffin, over the whole epidermis, including denuded areas
- Apply a topical antimicrobial agent to sloughy areas only.
- Silver-containing products/dressings may be considered
- The detached, lesional epidermis may be left in situ to act as a biological dressing. Blisters should be decompressed by piercing and expression or aspiration of tissue fluid
- Apply nonadherent dressings to denuded dermis
- A secondary foam or burn dressing should be used to collect exudate
Transfer to a Burn Centre in patients with TEN (> 30% BSA epidermal loss) and evidence of ANY of the following:
- Clinical deterioration,
- Extension of epidermal detachment,
- Subepidermal pus,
- Local sepsis,
- Wound conversion,
- Delayed healing.
In a burn center, conservative measures may be supplemented with a surgical approach:
- Remove necrotic/loose infected epidermis and clean wounds using a topical antimicrobial agent (e.g. betadine or chlorhexidine) under general anaesthetic
- Consider debridement with VersajetTM
- Physiological closure with Biobrane/allograft/xenograft skin in patients with early presentation involving noninfected and large confluent areas
7. Fluid Replacement Regimen:
- IV lines inserted through nonlesional skin, whenever possible, and change venous cannulas every 48 h
- Monitor fluid balance carefully: catheterize if appropriate/necessary
- Establish adequate IV fluid replacement initially. Fluid replacement:
- Is guided by urine output and other endpoint measurements.
- Should be adjusted on a daily basis
- Oral administration of fluids with the improvement of SJS/TEN mouth involvement
7. Nutrition Regimen:
- Provide continuous enteral nutrition throughout the acute phase
- Deliver up to 20–25 kcal/kg daily during the early, catabolic phase, and 25–30 kcal/ kg daily during the anabolic, recovery phase
- Use a patient-appropriate validated pain tool to assess pain in all conscious patients at least once daily
- Patients should receive adequate analgesia to ensure comfort at rest, with the addition of supplementary opiates, as required
- Additional analgesia may be needed to address increased pain associated with patient handling, repositioning, and dressing changes
9. Supportive therapeutic measures:
- Immobile patients should receive low molecular weight heparin
- Patients in whom enteral nutrition cannot be established should receive a proton pump inhibitor to reduce the risk of stress-related gastrointestinal ulceration
- Neutropenic patients may benefit from recombinant human G-CSF
10. Treatment of Eye involvement:
- Daily ophthalmological review during the acute illness
- Apply an ocular lubricant (e.g. nonpreserved hyaluronate or carmellose eye drops) every 2 h through the acute illness
- Ocular hygiene must be carried out each day by an ophthalmologist or ophthalmically trained nurse
- Application of topical corticosteroid drops (e.g. nonpreserved dexamethasone 0.1% twice daily) may reduce ocular surface damage
- Administer a broad-spectrum topical antibiotic as prophylaxis (e.g. moxifloxacin drops four times daily) in the presence of corneal fluorescein staining or frank ulceration
- In the unconscious patient, prevention of corneal exposure is essential
11. Treatment of mouth involvement:
- Daily oral review is necessary during the acute illness
- Apply white soft paraffin ointment to the lips every 2 h through the acute illness
- Clean the mouth daily with warm saline mouthwashes or an oral sponge
- Use an anti-inflammatory oral rinse or spray containing benzydamine hydrochloride every 3 h, particularly before eating
- Use an antiseptic oral rinse containing chlorhexidine twice daily
- Use a potent topical corticosteroid mouthwash (e.g. betamethasone sodium phosphate) four times daily
12. Treatment of Urogenital involvement:
- Daily urogenital review is necessary during the acute illness
- Apply white soft paraffin ointment to the urogenital skin and mucosae every 4 h through the acute illness
- Use a potent topical corticosteroid ointment once daily to the involved, but noneroded, surfaces
- Use a silicone dressing (e.g. MepitelTM) to eroded areas
13. Treatment of airway involvement:
Respiratory symptoms and hypoxaemia on admission should prompt early discussion with an intensivist and rapid transfer to an ICU or burn centre, where fibre optic bronchoscopy should be undertaken
14. Active therapy:
If active therapy is instituted it should be given, ideally, under the supervision of a specialist skin failure MDT in the context of clinical research and/or case registry
15. Discharge and follow-up:
- Give the patient written information about the drug(s) to avoid
- Encourage the patient to wear a MedicAlert bracelet
- Drug allergy should be documented in the patient’s notes;
- All doctors involved in the patient’s care should be informed
- Report the episode to the national pharmacovigilance authorities
- Organize a dermatology outpatient clinic appointment, and, if required, an ophthalmology outpatient appointment, within a few weeks of discharge
- Refer for review to the unit with the appropriate sub-specialty interest
16. Diagnostic Testing:
- Routine drug hypersensitivity testing is not recommended following an episode of SJS/TEN
- Seek specialist advice on hypersensitivity testing when
- Culprit drug is not known, or
- Medication avoidance is detrimental to the individual, or
- Accidental exposure is possible.
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Dr. Kamal Kant Kohli
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