Stevens-Johnson Syndrome

Incidence of SJS and TEN, a more severe variant of SJS, is 2–7 cases per million per y.

Incidence around 100 times higher in the HIV-positive population.

More common in women.

Affects all age groups.

High risk for infection

Hypovolemia

Cutaneous, mucosal, and ocular injury

Respiratory failure requiring mechanical ventilation in around 25% of pts

Sepsis and septic shock

Fluid and lyte imbalances

Development of multiorgan failure

Disease recurrence if culprit drug is readministered

Severe cutaneous reaction with epidermal necrosis and detachment in conjunction with mucosal and conjunctival involvement.

SJS and TEN fall along a disease continuum. SJS is less severe, involving <10% total BSA. TEN involves >30% BSA, and SJS-TEN overlap involves 10–30%.

Clinical presentation:

• Prodrome: Fever, flu-like symptoms (malaise, myalgia, arthralgia), skin pain/tenderness, oral pain, photophobia, and conjunctival burning can be early signs of mucosal involvement.

• Cutaneous lesions: Diffuse erythema or erythematous macules starting on trunk and face and developing central necrosis and bullae formation with eventual sloughing off of epidermis and exposed dermis.

• Mucosal involvement in 90% of pts.

Mortality from SJS is around 5–10% and increases to 30% or more for TEN.

Mortality primarily from sepsis, respiratory failure, and multiorgan dysfunction.

• Prognosis worse with advanced age and greater BSA involvement.

• Prognostic scoring system, called SCORTEN, can estimate pt survival.