Stevens-Johnson Syndrome (SJS)

Stop the drug and never give it again - Discontinuing the culprit within the first 24 hours of blister formation lowers infection rate, shortens stay, and improves survival. Get the drug and its whole class into the chart, the wristband, and your own head before you open a syringe: 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. Cross-reactivity within a class is reported, so structurally related drugs are out too. This is the single most consequential thing you will do for this patient.

The threshold, and how to count it - Under 10% detached body surface is SJS, 10 to 29% is overlap, over 30% is TEN. Only detached skin — blisters and erosions — or potentially detachable skin with a positive Nikolsky sign counts. Erythema alone does not. Blistering over more than 10% of the surface usually means ICU or burn unit admission.

Mucosal involvement is your airway problem - Two or more mucous membranes are involved in about 90% of SJS and TEN. Lips, mouth, pharynx, esophagus, GI tract, eyes, genitals, and the upper respiratory tract all ulcerate and erode. Expect a mouth that bleeds when you touch it. Lubricate well, go a size down on the tube, and have suction ready for blood. Reach for the video laryngoscope rather than levering a blade against friable tissue — in predicted difficult airways it delivers improved laryngeal views, more successful and more first-attempt intubations, and fewer maneuvers than direct laryngoscopy (2022 ASA difficult airway guidelines).

Airway mucosa can slough - Respiratory tract involvement is described and can produce serious pulmonary disease even with a normal chest radiograph, and pulmonary sloughs are what the pulmonary toilet consults exist for. If there is respiratory distress, intubate — but let the most experienced operator do it, and expect secretions and shed mucosa to threaten the tube.

Nothing adhesive, anywhere - Skin care in these patients means non-adherent dressings and avoidance of trauma to the skin, and that includes everything anesthesia routinely sticks on. Adhesive ECG electrodes, tape on the tube and the eyelids, and the repeated shear of a cycling blood pressure cuff all create fresh erosions on skin that is already detaching. Use needle or clip electrodes, tie or suture the tube, site the cuff over intact skin, and consider an arterial line if the case justifies one.

Treat it like a burn - Fluid resuscitation with crystalloid guided by a standard burn 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.

Temperature - The barrier is gone and heat goes with it. Prevention of hypothermia starts in the prehospital setting and does not stop in your room: warm the room ahead of time, forced air over intact skin, warmed fluids.

Eyes - Ocular disease is common and among the worst long-term sequelae, running from conjunctivitis and eyelid edema to corneal ulceration and blindness. Do not tape the lids. Lubricate, and get ophthalmology in early — treatment is topical lubricants, antibiotics, or steroid drops, with amniotic membrane transplantation used for severe acute ocular involvement.

Bleeding with mucosal disease - Keep the coagulation profile and blood count in normal range in anyone with extensive mucosal involvement; they can bleed severely, particularly from the GI tract. Anemia and lymphopenia are common, and neutropenia is an unfavorable prognostic sign. Transfusion of blood or products may be needed.

Score it with SCORTEN - Seven variables in 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 3.2% at 0 to 1 points, 12.1% at 2, 35.3% at 3, 58.3% at 4, and over 90% at 5. Mortality is more than 40 times higher with a bicarbonate under 20 mmol/L.

Infection is what kills, not the rash - Sepsis is the most common serious acute risk. Prophylactic antibiotics are not recommended and do not change survival. Skin lesions get microbiologic swabs 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 - These patients are anxious and hurting badly, and the pain often precedes the visible eruption. Opiates or PCA are the standard, and good wound care itself cuts the analgesic requirement.

Debridement goes under general anesthesia - When the surgical team debrides, it is a general anesthetic, and it is the most likely reason you will meet these patients. Run it as a burn case: warm room, monitoring and access sited through intact skin, blood available.

Feeding tube - Enteral nutrition beats parenteral. A nasogastric tube is used when the oral mucosa is significantly involved; pass it gently, because the same eroded mucosa continues down the esophagus.

Pharmacotherapy is unsettled - No treatment is proven. Systemic corticosteroids are often given in high doses for the first three to five days but their benefit is unknown, and ciclosporin, TNF-alpha inhibitors, N-acetylcysteine, plasmapheresis, and IVIG all remain controversial. Do not build your plan around any of them.

Not erythema multiforme, not SSSS - Erythema multiforme major involves under 10% of body surface with symmetric acral target lesions; SJS and TEN are blisters on erythematous macules in a central, facial and truncal distribution with mucosal involvement in 90%. Staphylococcal scalded skin syndrome spares mucosa entirely and splits the epidermis superficially. Skin biopsy distinguishes them, and the answer changes the plan.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are one disease separated only by how much skin has detached: under 10% of total body surface area is SJS, 10 to 29% is SJS/TEN overlap, and over 30% is TEN. A drug causes over 80% of cases.

Drug-specific CD8+ cytotoxic T cells drive keratinocyte apoptosis through the Fas-Fas ligand pathway, granule-mediated exocytosis, and TNF-alpha; histology shows keratinocyte and epidermal necrosis with only a mild dermal lymphocytic infiltrate and negative direct immunofluorescence. Onset is a few days to eight weeks after starting the drug, and a repeat exposure can produce symptoms within hours. SJS affects roughly 9.3 per million people per year and carries about 1 to 5% mortality — but the count you take today may not be the count tomorrow, because SJS can march into TEN.