First described in 1922, Stevens-Johnson syndrome (SJS) is an immune-complex–mediated hypersensitivity complex that is a severe expression of erythema multiforme. It is known by some as erythema multiforme major, but disagreement exists in the literature. Most authors and experts consider Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) different manifestations of the same disease. For that reason, many refer to the entity as SJS/TEN. SJS typically involves the skin and the mucous membranes. While minor presentations may occur, significant involvement of oral, nasal, eye, vaginal, urethral, GI, and lower respiratory tract mucous membranes may develop in the course of the illness. GI and respiratory involvement may progress to necrosis. SJS is a serious systemic disorder with the potential for severe morbidity and even death. Missed diagnosis is common.
Although several classification schemes have been reported, the simplest breaks the disease down as follows :
* Stevens-Johnson syndrome - A "minor form of TEN," with less than 10% body surface area (BSA) detachment
* Overlapping Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) - Detachment of 10-30% BSA
* Toxic epidermal necrolysis - Detachment of more than 30% BSA
Paramedics should recognize the presence of severe fluid loss and should treat patients with Stevens-Johnson syndrome (SJS) as they would patients with thermal burns.
Emergency Department Care
Most patients present early and prior to obvious signs of hemodynamic compromise. The single most important role for the ED physician is to detect Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) early and initiate the appropriate ED and inpatient management.
Withdrawal of the suspected offending agent is critically important. Timing of withdrawal has been linked to outcome.
* Care in the ED must be directed to fluid replacement and electrolyte correction.
* Skin lesions are treated as burns.
* Patients with SJS/TEN should be treated with special attention to airway and hemodynamic stability, fluid status, wound/burn care, and pain control.
* Treatment is primarily supportive and symptomatic. Some have advocated corticosteroids, cyclophosphamide, plasmapheresis, hemodialysis, and immunoglobulin.
o Manage oral lesions with mouthwashes.
o Topical anesthetics are useful in reducing pain and allowing the patient to take in fluids.
o Areas of denuded skin must be covered with compresses of saline or Burow solution.
o Address tetanus prophylaxis.
* Underlying diseases and secondary infections must be identified and treated. Offending drugs must be stopped.
* The use of systemic steroids remains controversial. Some authors believe that they are contraindicated, especially because there may be some question about the diagnosis. Patients with infection-induced erythema multiforme do worse when steroids are given. (Note that the differentiation between SJS and EM should be possible even in the acute stage.) Treatment with systemic steroids has been associated with an increased prevalence of complications. The ophthalmology literature contains several papers that advocate systemic and topical steroids to minimize ocular morbidity. Authors have cited sight salvage when pulse steroid therapy has been given. Others have concluded that IV steroids and immunoglobulin treatment do not improve outcome.18 The ophthalmology literature also mentions concurrent coverage of the involved eye(s) with amniotic membrane.
* In a large European study designed to evaluate the efficacy of various treatments, the EuroSCAR Study "found no sufficient evidence of a benefit for any specific treatment."20 The group looked at mortality in patients treated with IV immunoglobulins and corticosteroids. However, in a letter to the editor, Pehr disagreed with the findings in the EuroSCAR study citing inadequate doses of IVIG and corticosteroids in that study.
No specific drug treatment has been consistently shown to be beneficial in the treatment of Stevens-Johnson syndrome. The choice of antibiotic for infectious causes depends on the cause of that infection.
Clinical and laboratory evidence suggesting bloodstream infection mandates the use of antibiotics. The most common organisms include Staphylococcus aureus, Pseudomonas aeruginosa, and Enterobacteriaceae species.
The use of systemic corticosteroids is controversial, but may be useful in high doses early in the disease. Morbidity and mortality actually may increase in association with corticosteroid use.
Human intravenous immunoglobulin has been described as both treatment and prophylaxis. In the latter setting, one group used IVIG in a patient who underwent cardiac catheterization but who had 4 previous Stevens-Johnson syndrome (SJS) episodes after intravenous contrast injection.