Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Diagnosis and Management

Clinical Presentation and Diagnostic Features

The clinical presentation of large strips of skin peeling off the feet and lower legs leaving full-thickness ulcers, combined with platelet clumping on CBC, is most consistent with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) 1, 2
Extensive epidermal detachment occurs as sheets of necrotic epidermis separate from underlying dermis, leaving exposed denuded dermis 2, 3
Lesional skin is extremely tender to touch, and minimal shearing forces cause the epidermis to peel back (positive Nikolsky sign) 1, 2, 3
Flaccid bullae develop as necrotic epidermis separates, progressing to large areas of confluent erythema in severe cases 1, 2
The denuded dermis exudes serum, becomes secondarily infected, and readily bleeds 1, 3

Platelet clumping on CBC can occur as part of the systemic inflammatory response and hematologic complications 4
Granulocytopenia (low white blood cell count) is associated with worse prognosis 4
Anemia and leucopenia are common sequelae of acute skin failure 4
Calculate SCORTEN within the first 24 hours of admission to predict mortality risk 4
SCORTEN uses seven clinical parameters including age, BSA involvement, presence of septicemia, and granulocytopenia 4

The extent of epidermal detachment determines classification and prognosis 5
SJS: <10% body surface area (BSA) detachment 3, 5
SJS-TEN overlap: 10-30% BSA detachment 3, 5
TEN: >30% BSA detachment 3, 5
Patients with >10% BSA epidermal loss must be admitted without delay to a burn center or ICU with experience treating SJS/TEN 4, 5
Delayed transfer to a specialist unit is associated with increased mortality 4
Barrier nursing in a controlled environment (25-28°C ambient temperature, pressure-relieving mattress) is essential 4

Coordinate care through dermatology/plastic surgery, intensive care, ophthalmology, and specialist nursing 4
Additional input may be needed from respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, and physiotherapy 4
Minimize shearing forces when moving and positioning patients, as necrotic epidermis is prone to detach 4
Careful antishear handling techniques are essential during bedside care 4

Document all medications (including over-the-counter preparations) taken over 2 months prior to symptom onset 4
Most common drug triggers include allopurinol, carbamazepine, lamotrigine, nevirapine, oxicam NSAIDs, phenobarbital, phenytoin, sulfamethoxazole, and sulfasalazine 4
In children, infection (particularly Mycoplasma pneumoniae) is more commonly the precipitant than drugs 1
Recurrence is more common in children (up to 18% of cases) when infection is the cause 1