Toxic Epidermal Necrolysis (TEN)

Stop the drug, and it can never come back - Discontinuing the culprit and getting the patient to a burn unit or an experienced ICU within the first 24 hours of blister formation lowers infection rate, shortens stay, and improves survival. Document the drug and everything structurally related, because cross-reactivity is reported: the Euro-SCAR high-risk list is lamotrigine, carbamazepine, phenytoin, nevirapine, phenobarbital, sulfonamides, sulfasalazine, allopurinol, and oxicam NSAIDs, and also implicated are chloramphenicol, penicillins, quinolones, barbiturates, valproate, oseltamivir, and abacavir. Never re-administer any of them to a survivor.

Same spectrum as SJS, split by body surface area - Under 10% detached is SJS, 10 to 29% is overlap, over 30% is TEN. Count only detached skin — blisters and erosions — or skin that peels under light pressure (positive Nikolsky sign). Erythema alone does not count. Progression is unpredictable: it usually takes two weeks but can take the whole body inside 24 hours.

The lesions start exactly where your equipment goes - The eruption begins on the face and presternal area before spreading over the torso, and the scalp is spared in almost all cases. That is your mask, your ECG leads, and your defib pads. Plan monitoring and securement around it; the spared scalp is one place that will still hold something.

Nothing adhesive, anywhere - Wound care here is non-adherent dressings and avoidance of skin trauma. Adhesive electrodes, tape on the tube and the eyelids, and the repeated shear of a cycling blood pressure cuff each create new erosions. Use needle or clip electrodes, tie or suture the tube, site the cuff over intact skin, and go arterial if the case justifies it.

Airway mucosa is involved and can slough - Mucosal erythema and erosion occur in 90% of cases and usually precede the skin eruption by 1 to 3 days. Oropharynx, eyes, and genitalia are the usual sites, but respiratory and GI tract involvement is described, and respiratory disease can be serious with a normal chest radiograph. If there is distress, intubate — but let your most experienced operator do it, because the tissue bleeds and shed mucosa can obstruct the tube. Keep suction, a smaller ETT, and a pulmonary toilet plan ready, and favor the video laryngoscope: in predicted difficult airways it gives improved laryngeal views, more successful and more first-attempt intubations, and fewer maneuvers than direct laryngoscopy (2022 ASA difficult airway guidelines).

Resuscitate like a burn - Crystalloid guided by a standard burn resuscitation formula such as Parkland, targeting a MAP above 65 mmHg, CVP 8 to 12 mmHg, and urine output 0.5 to 1 mL/kg/hr.

Hypothermia - The barrier is gone. Prevention starts before the patient reaches you and continues in your room: warm the room first, forced air, warmed fluids, non-adhesive sterile dressings over erosions.

Debridement is a general anesthetic - There is no consensus on early, late, or no debridement, but when it happens it is done under general anesthesia — most likely how you will meet this patient. Wounds get biologic (allograft, xenograft, homograft), biosynthetic, or silver-impregnated dressings afterward, and good wound care reduces the analgesic requirement.

Bleeding - Keep the coagulation profile and blood count within normal values, especially with extensive mucosal involvement, because these patients can bleed severely from the GI tract. Anemia and lymphopenia are common; neutropenia is an unfavorable prognostic sign. Transfusion of blood or products may be considered.

Infection is the main cause of death - Staphylococcal infection is most common, followed by pseudomonas after prolonged admission. Prophylactic antibiotics are not recommended and do not change survival. Swab skin lesions on presentation and every 48 hours; treat on a positive swab, rapid deterioration, or a sudden drop in temperature, covering gram-positives, gram-negatives, and anaerobes.

Pain - Severe, and it precedes the vesicular eruption. Opiates or PCA.

Score it with SCORTEN - Seven variables inside the first 24 hours: age over 40, heart rate 120 or more, cancer or hematologic malignancy, detached body surface area of 10% or more on day one, BUN over 28 mg/dL, bicarbonate under 20 mEq/L, and glucose over 252 mg/dL. Mortality runs from 3.2% with a single risk factor to over 90% with five or more.

Eyes - Ocular involvement is among the most common and most serious sequelae: corneal ulcer, xerophthalmia, meibomian gland dysfunction, panophthalmitis, blindness. Do not tape the lids. Lubricate, and get ophthalmology in on day one — topical lubricants, antibiotics, or steroid drops, with amniotic membrane transplantation for severe acute disease.

Nutrition - TEN is hypercatabolic. Enteral feeding beats parenteral because it reduces bacterial translocation, aiming for 20 to 25 kcal/kg/day, with a nasogastric tube when the oral mucosa is badly involved.

Survivors carry strictures - Healed mucosa scars into stenoses and strictures — esophageal, genitourinary, and gingival synechiae — and xerostomia follows salivary gland involvement. A survivor coming back for an unrelated case may have a narrowed airway or esophagus, so ask about swallowing and hoarseness and size the tube down.

Pharmacotherapy is unproven - No modality is established. Systemic steroids are commonly used but some studies link them to increased mortality; plasmapheresis, ciclosporin, TNF-alpha inhibitors, and IVIG are all supported only by observational data. Do not build your plan around any of them.

Not staphylococcal scalded skin syndrome - SSSS looks similar but splits the epidermis superficially, is toxin-mediated, and spares mucosa. TEN necroses the full epidermis and involves mucosa in 90% of cases. Skin biopsy with immunofluorescence separates them, and the answer decides whether you are running a burn-type resuscitation with a sloughing airway or treating a staph infection.

Toxic epidermal necrolysis (TEN), originally Lyell syndrome, is the severe end of the Stevens-Johnson syndrome (SJS)/TEN spectrum — the same drug-induced epidermolysis, divided only by detached body surface area. Under 10% is SJS, 10 to 29% is overlap, and over 30% is TEN.

Keratinocytes undergo full-thickness necrosis and the epidermis separates from the dermis, with strikingly little inflammatory infiltrate. Fas-Fas ligand and granulysin-mediated apoptosis are implicated, with reactive oxygen species proposed as the initiating insult when the culprit drug overwhelms detoxification pathways. Drugs cause most cases, usually 1 to 3 weeks after starting; infection, malignancy, and vaccination account for the rest, and about 5% have no recent drug exposure at all. TEN is rare at roughly 1.9 per million adults per year, but mortality is 25 to 30% — infection and multiorgan failure, not the skin itself, are what kill.