Table of Contents
ToggleLearning objectives
- Define TEN and place it on the Stevens–Johnson syndrome (SJS) spectrum.
- Recognise the prodrome, painful rash, epidermal detachment and multisite mucosal disease.
- Perform an ABCDE assessment while protecting the airway, skin barrier, eyes, fluids and temperature.
- Identify culprit medicines, stop unsafe exposures and document a permanent severe drug reaction.
- Use a structured approach to fluids, wound care, analgesia, nutrition, infection surveillance and organ support.
- Explain when to transfer to ICU/burns care and how to prevent long-term ocular, genital, pulmonary and psychological complications.
Definition and SJS–TEN spectrum
SJS and TEN are severe, immune-mediated epidermal necrolysis syndromes. They are classified by the percentage of detached or detachable body-surface area (BSA): SJS is generally less than 10%, SJS–TEN overlap is 10–30%, and TEN is more than 30%. The cutaneous percentage does not fully describe severity because small areas with extensive mucosal disease can threaten the airway, eyes, nutrition and urine flow. A positive Nikolsky sign (epidermis shearing with gentle pressure) may occur, but forceful rubbing is unsafe.
The condition is not ordinary urticaria, a simple drug rash or an infection that can be managed with topical cream. It is a time-critical dermatological and resuscitation emergency and should be managed in hospital, preferably in a burns or intensive-care environment when extensive.
Causes and high-risk medicines
Most adult cases are medication-related. The reaction often begins one to four weeks after a new medicine, but it can occur sooner after re-exposure or later with some agents. Commonly implicated groups include:
- Allopurinol.
- Sulfonamide antibiotics, including co-trimoxazole, and other antibiotics.
- Aromatic anticonvulsants such as carbamazepine, phenytoin, phenobarbital and lamotrigine.
- Some non-steroidal anti-inflammatory drugs, particularly oxicam derivatives.
- Nevirapine and other antiretroviral or immunomodulating medicines.
- Other drugs, herbal preparations and combinations must be assessed individually; never assume an unfamiliar medicine is harmless.
Infections such as Mycoplasma pneumoniae and other triggers may produce a similar mucocutaneous syndrome, especially in younger people. Ask about recent infections, vaccines, cancer therapy, radiotherapy and herbal or traditional medicines.
Pathophysiology
- Cytotoxic T-cell and natural-killer-cell responses injure keratinocytes through granulysin, perforin/granzyme and related pathways.
- Widespread apoptosis causes full-thickness epidermal necrosis and separation at the dermoepidermal junction.
- Loss of the epidermal barrier causes transepidermal water loss, hypothermia, pain, fluid and electrolyte disturbance and susceptibility to invasive infection.
- Mucosal epithelial necrosis causes conjunctivitis and corneal injury, painful oral erosions, dysphagia, genital/urinary erosions and airway or respiratory complications.
- Inflammatory mediators can produce renal, hepatic, pulmonary, cardiac and haematological organ dysfunction.
Early presentation and clinical progression
The first phase resembles a severe viral illness: fever, malaise, sore throat, cough, headache, myalgia, arthralgia and eye irritation. Within days, the patient develops skin pain and dusky erythematous or atypical targetoid macules, often on the trunk and face. Lesions coalesce, become flaccid blisters and peel, leaving raw, tender erosions. Mucosal involvement is common and may precede obvious skin detachment.
- Skin: severe tenderness, dusky macules, flaccid bullae, epidermal detachment, erosions and a positive gentle-shear sign.
- Eyes: red painful eyes, photophobia, discharge, blurred vision, conjunctival membranes or inability to open the eyes.
- Mouth and throat: painful lips, haemorrhagic crusting, oral erosions, drooling, dysphagia and inability to drink.
- Genital and urinary: erosions, dysuria, urinary retention, vaginal or urethral involvement.
- Respiratory: cough, hypoxia, wheeze, bronchial sloughing or respiratory failure.
- Systemic: tachycardia, fever or hypothermia, hypotension, dehydration, confusion and reduced urine output.
Recognition: red flags requiring immediate escalation
Do not wait for a dermatology appointment, a positive culture or a complete laboratory panel before stopping a suspected culprit and arranging transfer.
Immediate contact and safety
- Use standard precautions, hand hygiene and gentle handling; add eye and body protection if there is fluid leakage.
- Place the patient in a warm, low-traffic area and minimise friction, adhesives and unnecessary transfers.
- Ask the patient or family to bring all medicines, blister packs, traditional remedies and recent prescriptions.
- Stop the suspected culprit immediately after a clinician review; stop non-essential medicines until the medication list is reconciled.
- Do not restart, rechallenge or give a related high-risk drug without specialist advice.
- Call dermatology, critical care/burns, ophthalmology and relevant surgical or urology/gynaecology teams early.
ABCDE assessment
A — Airway
- Listen for hoarseness, stridor, muffled voice, drooling or inability to swallow.
- Inspect oral and pharyngeal sloughing; ask about throat pain and aspiration.
- Prepare suction, oxygen and difficult-airway equipment. Involve anaesthesia early because progressive mucosal oedema and sloughing can make later intubation hazardous.
B — Breathing
- Measure respiratory rate, work of breathing, SpO2 and chest excursion; ask about cough or pleuritic pain.
- Give oxygen for hypoxaemia, obtain blood gas or chest imaging when indicated, and consider bronchoscopic assessment for airway sloughing in critical care.
C — Circulation
- Check pulse, blood pressure, capillary refill, peripheral temperature, mental status and urine output.
- Establish IV access through intact skin where possible; take blood samples, group and cross-match if extensive, and culture when infection is suspected.
- Replace fluid and electrolytes using careful reassessment rather than blindly applying a major-burn formula; TEN losses vary with detachment and urine output.
D — Disability
- Assess GCS/AVPU, glucose, pain, delirium and anxiety.
- Severe pain, hypoxia, sepsis, dehydration and opioid accumulation can all alter mental state.
E — Exposure
- Expose gently, inspect the entire skin and all mucosal sites, estimate detached/detachable BSA and prevent heat loss.
- Remove wet clothing and jewellery carefully; avoid adhesive ECG pads on denuded skin and use non-traumatic alternatives.
Focused history and medication reconciliation
| History area | Questions and emergency relevance |
|---|---|
| Timeline | When did each medicine start, stop or change? When did fever, pain, rash and mucosal symptoms appear? |
| All products | Prescription, over-the-counter, antimalarial, antibiotic, anticonvulsant, ART, herbal, traditional, topical and recreational products. |
| Previous reaction | Any prior rash, allergy, hospitalisation or family history of severe drug reactions? |
| Mucosal function | Can the patient see, swallow, drink, urinate and breathe normally? These answers guide urgent specialist care. |
| Comorbidity | HIV, cancer, renal/liver disease, diabetes, pregnancy, epilepsy, malnutrition or immune suppression. |
| Exposure/infection | Recent respiratory illness, sick contacts, travel, vaccination or radiotherapy. |
Record generic and brand names, dose, route, dates and indication. If several medicines were started together, do not guess the culprit: stop unnecessary drugs, seek expert review and report the suspected adverse reaction through the appropriate pharmacovigilance system.
Examination and estimating skin involvement
- Describe lesion type, colour, tenderness, blistering, detachment and distribution. Document whether skin is intact, detachable or already denuded.
- Estimate BSA using the patient’s palm including fingers as approximately 1% or a locally taught chart; record the method used.
- Assess the mouth, eyes, nose, ears, genitalia and anus while protecting privacy.
- Check for pressure injury, infection, purulence, malodour, necrosis, peripheral perfusion and joint movement.
- Repeat the examination because detachment can progress after arrival.
Differential diagnoses
| Condition | Distinguishing clues | Immediate implication |
|---|---|---|
| Staphylococcal scalded skin syndrome | Usually children, superficial tenderness and positive Nikolsky but mucosa often spared. | Urgent paediatric/infectious-disease care; culture suspected focus. |
| Generalised bullous fixed drug eruption | Fewer sharply demarcated lesions, recurrent at same sites and less systemic illness. | Still stop culprit; specialist confirmation needed. |
| Erythema multiforme | Typical raised target lesions, often acral and less extensive detachment. | Assess mucosa and rule out SJS/TEN when severe. |
| Acute generalized exanthematous pustulosis | Numerous sterile pustules, neutrophilia and shorter course. | Stop culprit and monitor systemic complications. |
| Generalised drug eruption | Morbilliform rash without skin pain, detachment or major mucosal erosions. | May be less dangerous, but reassess if it evolves. |
| Burn or chemical injury | Exposure history and distribution matching contact; no typical drug latency. | Remove exposure and use burn protocol. |
| Necrotising infection | Severe focal pain, toxicity, gas or purulence rather than diffuse mucocutaneous necrolysis. | Urgent surgical review and antimicrobials. |
Investigations and diagnosis
- Full blood count, electrolytes, urea/creatinine, glucose, liver tests, bicarbonate and albumin.
- Blood gas and lactate if shock, hypoxia or organ dysfunction is present.
- Blood cultures and cultures of clinically infected sites; do not give prophylactic antibiotics simply because skin is denuded.
- Urinalysis and urine culture if dysuria, retention or sepsis is suspected.
- Chest radiograph, ECG and additional imaging guided by respiratory or organ symptoms.
- Skin biopsy from a fresh lesion for histopathology and direct immunofluorescence when available; treatment should not wait for the report.
- Use SCORTEN or the locally adopted prognostic tool within the first 24 hours and repeat at the recommended interval. It supports communication and escalation; it does not replace bedside judgement.
Immediate treatment priorities
- Stop the suspected drug: the most important time-critical intervention.
- Transfer: arrange admission to ICU, a burns unit or a specialist centre according to BSA, physiology and local capability.
- Protect the barrier: use a warm environment, pressure-relieving surface, non-adherent dressings and gentle handling.
- Control pain: titrate appropriate analgesia, monitor sedation and avoid unnecessary intramuscular injections through damaged skin.
- Replace losses: give warmed fluids and electrolytes based on perfusion, urine output, serum results and ongoing loss.
- Assess mucosa early: urgent ophthalmology for every patient with eye symptoms; assess mouth, airway, genital and urinary involvement.
- Prevent secondary harm: avoid adhesive tape, friction, unneeded antibiotics, latex exposure and unreviewed medicines.
Skin and wound care
- Use a warm room and a pressure-relieving mattress; minimise handling, shear and unnecessary dressing changes.
- Clean gently with sterile saline or an approved non-irritating solution. Avoid vigorous scrubbing, alcohol and harsh antiseptics on denuded tissue.
- Protect detached areas with non-adherent silicone or paraffin gauze and an appropriate secondary dressing; follow the burns/dermatology team’s method.
- Leave stable epidermal roofs in place unless they are infected, non-viable or obstruct care; debride only with expert direction.
- Elevate oedematous limbs, protect heels and reposition carefully.
- Use aseptic technique, document drainage and inspect for increasing pain, pus, odour, necrosis or new systemic deterioration.
Fluid, electrolyte and temperature management
TEN patients lose fluid through damaged skin and may be unable to drink because of oral erosions. Over-resuscitation can cause pulmonary oedema, while under-resuscitation causes shock and kidney injury. Use warmed isotonic fluid, frequent reassessment and a urinary catheter only when indicated and managed aseptically.
- Set a urine-output target with the senior team and monitor hourly in unstable or extensive disease.
- Record all oral, IV, enteral and urinary losses; trend weight, oedema, sodium, potassium, bicarbonate, creatinine and albumin.
- Correct electrolyte abnormalities gradually and safely; review renal function before nephrotoxic medicines.
- Prevent hypothermia with a warm room, warmed fluids, blankets and minimal exposure time.
- Review fluid needs whenever skin loss changes, fever rises, oral intake improves or organ function worsens.
Mucosal and organ-specific care
Eyes
- Urgent ophthalmology assessment is required even when eye involvement looks mild.
- Use prescribed preservative-free lubricants, regular inspection and specialist-directed topical therapy; watch for epithelial defects, symblepharon, ulceration and visual loss.
- Do not forcibly open adhered eyelids or put unprescribed drops on a damaged cornea.
Mouth and nutrition
- Assess swallowing, hydration, oral bleeding and airway protection.
- Use gentle oral care, prescribed analgesic mouth preparations and soft, high-calorie foods or enteral feeding when safe.
- Involve dietetics, speech/swallow specialists and anaesthesia if aspiration risk is present.
Genital and urinary tract
- Inspect for erosions, retention, bleeding and adhesions with consent and a chaperone.
- Seek urology or gynaecology input; catheterisation may be necessary for retention but must be gentle and aseptic.
Respiratory system
- Monitor cough, oxygenation, voice, secretions and work of breathing. Sloughing can affect the tracheobronchial tree.
- Escalate for bronchoscopy, ventilatory support or airway intervention when clinically indicated.
Pain, anxiety and psychological support
- Assess pain at rest and during dressing changes; pre-medicate before procedures and use multimodal analgesia.
- Monitor opioid effect, respiratory rate, sedation, constipation and renal/hepatic considerations.
- Explain each touch and procedure, involve a trusted support person with consent and provide privacy.
- Offer psychological support; fear, isolation, body-image change and sleep disruption are common.
- Use physiotherapy and gentle range-of-motion exercises when medically safe to reduce contractures and pneumonia.
Infection prevention and treatment
Denuded skin is not automatically infected. Routine prophylactic systemic antibiotics are generally avoided because they select resistant organisms and can cause new drug reactions. Use meticulous hand hygiene, aseptic wound care, surveillance cultures only when indicated and prompt targeted therapy for clinical sepsis or a documented infection.
- Monitor temperature, haemodynamics, mental state, wound appearance, white-cell count, lactate and organ function.
- Look for increasing wound pain, purulence, malodour, cellulitis, respiratory infection, line infection or urinary infection.
- Take cultures before antibiotics when feasible, but do not delay treatment in shock.
- Use isolation according to local infection-prevention policy, especially if a transmissible infection is suspected.
- Review every medicine for unnecessary antimicrobial exposure and allergy risk.
Specialist-directed immunomodulatory therapy
No single systemic medicine has universal evidence or replaces withdrawal of the culprit and high-quality supportive care. Dermatology and critical-care teams may consider corticosteroids, ciclosporin, intravenous immunoglobulin or other immunomodulators based on disease timing, contraindications, local expertise and available evidence. Explain uncertainty, monitor infection and organ function, and never delay transfer while debating a specialist therapy.
Nursing care plan
| Problem | Goals | Core nursing actions |
|---|---|---|
| Impaired skin integrity | Protect tissue and support re-epithelialisation. | Warm environment, non-adherent dressings, aseptic technique, pressure relief and wound documentation. |
| Fluid/electrolyte loss | Stable perfusion, urine output and laboratory values. | Hourly input/output when indicated, daily weight, warmed fluids, laboratory trends and escalation of oliguria. |
| Severe pain | Comfort at rest and during care. | Regular pain scoring, pre-medication, gentle handling, prescribed analgesia and reassessment. |
| Risk of infection | Early recognition without unnecessary antibiotics. | Hand hygiene, aseptic lines/dressings, surveillance of wounds and rapid culture/escalation for sepsis. |
| Mucosal dysfunction | Maintain vision, airway, hydration, nutrition and urine flow. | Eye/oral/genital care as prescribed, swallow assessment, nutrition support and specialist referrals. |
Complications
- Hypovolaemic or distributive shock, acute kidney injury and electrolyte abnormalities.
- Sepsis, pneumonia, bloodstream infection and multiorgan failure.
- Airway obstruction, bronchial sloughing, hypoxaemia and respiratory failure.
- Corneal ulceration, symblepharon, scarring, dry eye and permanent visual loss.
- Oral strictures, malnutrition, genital adhesions, urethral stenosis and sexual dysfunction.
- Thromboembolism, pressure injury, contractures, chronic pain, pigment change and post-traumatic stress.
- Death; risk is related to age, extent, comorbidity, organ failure and delayed withdrawal of the culprit.
Transfer and escalation criteria
- Any suspected TEN or SJS with progressive detachment requires hospital admission and specialist consultation.
- Transfer to ICU/burns care for extensive BSA, shock, hypoxia, airway concern, severe mucosal disease, renal failure, altered mental state or rapidly worsening skin loss.
- Arrange specialist eye assessment for any ocular symptom and surgical/urology/gynaecology review for urinary or genital obstruction.
- Use a structured handover: culprit medicines and time, BSA, mucosae involved, airway, fluids, urine output, investigations, cultures, antibiotics and outstanding referrals.
Discharge, recovery and long-term follow-up
- Provide written documentation naming the suspected culprit and related medicines to avoid; add an allergy alert to the medical record and advise a medical-alert card or bracelet.
- Refer for ophthalmology follow-up even after eye symptoms improve; late scarring can appear after discharge.
- Review skin healing, pain, pigmentation, itch, nutrition, mobility, genital/urinary function and psychological wellbeing.
- Teach the patient to seek urgent care for fever, new blisters, eye pain, breathing difficulty, inability to drink or pass urine.
- Report the suspected adverse drug reaction to the national pharmacovigilance system and communicate it to every future healthcare provider.
Scenario-based application
Common errors to avoid
- Continuing the suspected culprit while waiting for a biopsy or specialist review.
- Calling the condition a simple allergy despite skin pain, blistering or mucosal erosions.
- Using routine prophylactic antibiotics on every denuded patient.
- Underestimating fluid, heat and nutritional losses because the visible BSA seems small.
- Applying adhesive dressings, vigorous debridement, alcohol or irritating antiseptics to fragile skin.
- Missing eye, airway, urinary or genital disease.
- Starting immunomodulatory therapy without specialist discussion or infection surveillance.
- Failing to document and communicate the culprit drug, leading to dangerous re-exposure.
Documentation checklist
- All medicines and products, start dates, suspected culprit and withdrawal time.
- ABCDE findings, vitals, pain, mental state, glucose, BSA estimate and detached versus detachable skin.
- Every mucosal site examined and specialist referrals made.
- Fluids, urine output, electrolyte trends, analgesia, dressings, cultures and antibiotics.
- SCORTEN or local severity assessment and transfer/handover details.
- Adverse-drug-reaction report, allergy alert, patient education and follow-up plan.
Quick revision questions
- How is TEN defined in relation to SJS and SJS–TEN overlap?
- Which medication groups commonly trigger TEN?
- What are the first three actions when TEN is suspected?
- Why must eyes, mouth, airway and genital/urinary mucosa be assessed early?
- Why is routine prophylactic antibiotic therapy discouraged?
- What factors determine transfer to a burns or intensive-care unit?
- What information must be written in the permanent drug-allergy record?
- List five complications that may persist after the skin heals.
Key takeaways
- TEN is a medical emergency, not a routine rash.
- Stop the suspected culprit immediately and arrange specialist hospital care.
- ABCDE, airway planning, warmed fluid/electrolyte care, analgesia and barrier protection save lives.
- Ophthalmology, nutrition, wound, urinary, respiratory and psychological care are essential—not optional extras.
- Prevent re-exposure by documenting, reporting and communicating the suspected medicine forever.