SJS & TEN
Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are a group of severe, life-threatening skin reactions in which the epidermis of the skin and mucous membranes dies and peels away over large areas. They are among the most urgent emergencies in dermatology and are usually caused by a delayed immune reaction to certain medications.
At Haute-Derma, we approach SJS/TEN with the mindset of an intensive care unit. Our protocol focuses on confirming the diagnosis early, stopping the culprit drug immediately and providing life support, so that as little skin as possible is lost and multi-organ failure is prevented.
Classification by Extent of Skin Detachment
At Haute-Derma, we grade severity by the percentage of body surface area (BSA) with detached skin:
- Stevens-Johnson syndrome (SJS): skin detachment of less than 10% BSA.
- SJS/TEN overlap: skin detachment of 10–30% BSA.
- Toxic epidermal necrolysis (TEN): skin detachment of more than 30% BSA.
Our Approach to Treating SJS/TEN
Our specialists at Haute-Derma know that every hour of delay raises the risk of death. We use the SCORTEN score to estimate severity and decide on the best resuscitation plan for each patient.
We focus on protecting the skin barrier and the mucous membranes (eyes, mouth, genitals) to prevent permanent complications such as blindness or scarring and narrowing of mucosal surfaces. At Haute-Derma, wound care is carried out under strictly sterile conditions and combined with the most up-to-date immunomodulatory therapies.
Emergency management includes:
- Stopping all suspected drugs immediately: the single most important step for improving the outlook.
- Fluid and electrolyte resuscitation: replacing fluid lost through raw, eroded skin.
- Systemic immunotherapy: IVIG or cyclosporine to halt skin cell death.
How We Diagnose SJS/TEN at Haute-Derma
To reach a diagnosis quickly, Haute-Derma carries out a thorough clinical and laboratory work-up:
- Recognizing early signs: high fever, sore throat and flu-like fatigue, followed by dusky red patches and intense burning pain in the skin.
- Checking for Nikolsky's sign: a characteristic sign in which light pressure on the skin makes the epidermis slide off the layer beneath.
- Assessing mucosal involvement: looking for erosions and crusting on at least two mucosal sites (eyes, mouth, nose or genitals).
- Rapid skin biopsy (frozen section): a frozen-section biopsy confirms full-thickness epidermal necrosis and distinguishes SJS/TEN from staphylococcal scalded skin syndrome (SSSS).
- SCORTEN scoring: calculated from age, heart rate, blood urea, blood glucose and the extent of skin involvement to predict outcome.
Specialized Treatment and Critical Care at Haute-Derma
Treatment at Haute-Derma is designed to shut down the immune "storm":
- Intravenous immunoglobulin (IVIG): blocks Fas–FasL signaling and so halts programmed cell death (apoptosis) in the epidermis.
- Cyclosporine: one of our first-line therapies at Haute-Derma; it suppresses cytotoxic T cells and significantly lowers mortality.
- Conservative wound care: we avoid rubbing or stripping away dead skin and use advanced biological dressings to create the right environment for the skin to regenerate.
- Multidisciplinary care:
- Ophthalmology: hourly eye care to prevent symblepharon (adhesions between the eyelid and eyeball) and corneal scarring.
- Nutrition: high-protein, high-calorie feeding given intravenously or through a feeding tube.
- Infection control: broad-spectrum antibiotics are used only when there is evidence of infection, to avoid making the drug reaction worse.
