Treatment

SJS is a serious systemic disorder with the potential for severe morbidity and mortality. In
approximately 5%–15% of cases SJS is fatal (35,39). In order to determine a patient’s risk of death with SJS,
clinicians bare encouraged to use the SCORTEN (TEN-specific severity of illness score) scale which employs
important prognostic indicators including heart rate, age, and renal function (91,92) (Table III). Assessing the
SCORTEN score requires giving the patient one point for each positive variable his condition fulfills. The total
points are tallied, with increased scores correlated with poorer prognosis.
Effective management of SJS begins with prompt recognition of the entity, combined with attention
to each of the major organs that may be affected, as well as potential comorbidities. Since medications are the
most common cause of SJS, a thorough drug history must be obtained, and all potential offending agents must
be immediately discontinued (32,39). Indeed, immediate cessation of involved medications appears to
improve the prognosis (93).
Comprehensive SJS treatment requires a skilled, collaborative, multidisciplinary approach that
addresses the highly complex, systemic response to the condition. A multispecialty team may also assist in
subsequent post discharge management including psychosocial issues that may arise from SJS-induced
disfigurations or scarring. The management of milder cases of SJS may occur in an inpatient ward with the
same fundamental therapeutic protocol used for burns: warming of the environment, minimizing
transepidermal water loss, treatment of electrolyte imbalances, administration of high-calorie nutrition and
intravenous fluids to prevent dehydration, and prevention of sepsis (38,39,94–96). For patients with
extensive cutaneous involvement, prompt referral to a burns unit has been shown to reduce the risk of
infection, mortality, and the length of hospitalization (94,96–101). This is particularly true for SJS caused by
drugs with short half-lives, which represent a positive prognostic factor for SJS (94). Patients must be
counselled regarding strict future avoidance of agents responsible for the outbreak as well as chemically
similar compounds. Given the suspected hereditary association with SJS, first-degree relatives should also be
encouraged to avoid similar chemical compounds.
Targeted nursing care including adequate maintenance of topical management reduces associated
morbidity and allows a more rapid reepithelialisation of skin lesions and the prevention of scarring, synechia
formation, and infection (39,100). Skin erosions should be covered with moisture-retentive ointments and/or
topical antibiotics to improve barrier function and to prevent bacterial infection. Given the involvement of the
lips and oral mucosa in many patients with SJS, adequate emphasis on alleviating pain associated with lesions
in the mouth is an integral part of treating the disorder. Application of petroleum jelly and sterile saline
compresses can promote rapid reepithelialization of the lips. The use of viscous lidocaine on the oral mucosa
in combination with diphenhydramine or sodium bicarbonate mouthwashes can dramatically relieve pain
associated with friable mucocutaneous ulcerations and prevent the onset of odynophagia (102–104).
For patients with ocular involvement, daily erythromycin eye drops are recommended to prevent
bacterial infections, and corticosteroid eye drops are administered to reduce inflammation (4). Continued
ophthalmologic care is recommended even after recovery in order to monitor and minimize irreversible
ocular complications including visual loss. Involvement of other organs is addressed with appropriate
supportive care as well as treatment of any strictures, adhesions, or scarring that may complicate the course
of the disease. Respiratory involvement may require prompt intubation and ventilatory support (28,29).
The use of medications to treat SJS has met with intense debate over the years. Treatment with
corticosteroids, while effective in most other acute inflammatory disorders, is controversial (105–110).
Furthermore, numerous other anti-inflammatory, immunosuppressive, and immunomodulatory agents, such
as cyclosporin, cyclophosphamide, thalidomide, and intravenous immunoglobulins (IVIg), have been
administered as possible means to arrest underlying immunological mechanisms promoting SJS
(39,111,112). However, the efficacy of these agents in the treatment of SJS has not been demonstrated by any
controlled clinical trial. In the absence of strong evidence, none of these regimens can be definitively
proposed as a treatment of choice. This notwithstanding, IVIg administered early after the onset of
mucocutaneous lesions is thought to hold the most promise for improvement in survival and a reduction in
longterm morbidity (82,83,113). The dose of IVIg administered varies, but typically is 1–3 g/kg/day for 3–5
days, with a mean total dose of 2.7 g/kg divided over 1–5 days (23,70,114,115). Studies have demonstrated
that IVIg arrests Fas-mediated keratinolysis in vitro, which provides a pathophysiologic explanation of why it
may improve SJS rough disruption of Fas-induced keratinocyte apoptosis (83,109). Moreover, intravenous
immunoglobulins have yielded promising results in controlled studies involving children (111) as well as
adults (115). For example, a recent retrospective analysis reported a 100% survival rate and complete skin
healing in 12 patients with SJS after treatment with IVIg (115). However, the absence of large or controlled
trials raises questions regarding the efficacy of IVIg, particularly given the high cost of this treatment (116). In
the absence of clear-cut benefits, potential risks of IVIg should be reviewed with the patient prior to
treatment.
Sepsis is a major source of mortality in SJS patients. However, prophylactic antibiotics are not
recommended in the treatment of acute SJS except when the etiologic agent is identified as an infectious
agent. For example, although supportive therapy was once considered treatment of choice for Mycoplasma-
induced SJS, antibiotic therapy is now typically administered to treat the infection (72). The presence of an
opportunistic infection due to HIV or immunosuppression represents a poor prognosis in the evolution of SJS
and toxic epidermal necrolysis (TEN) (36,37,40).
Although prophylactic antibiotics are not recommended, the use of skin cultures on the first day and
every 48 hours thereafter is recommended as a means of monitoring possible bacterial growth. Topical
antibiotic treatment should begin if there is an increased number of bacteria cultured from the skin with
selection of a single strain, a sudden drop in temperature, and/or deterioration in the patient’s condition.
Prompt and uninterrupted enteral nutrition reduces the incidence of stress ulcers and bacterial
translocation, and allows earlier discontinuation of intravenous lines (39). Finally, treating the patient’s
existing health concerns is paramount. For example, regardless of whether SJS is suspected to be secondary to
a drug reaction, prophylactic use of anticoagulants maybe indicated due to the risk of thromboembolism
induced morbidity and mortality (1). Furthermore, based on the severity of the disease, analgesics along with
supportive emotional and psychological care should be provided as needed.

Conclusions
SJS is a rare but serious adverse cutaneous reaction most commonly due to medications and infectious agents.
Prompt recognition is critical for the initiation of appropriate care. The mainstay of treatment remains
addressing the causative agent as well as supportive care for the mucocutaneous ulcerations. This requires a
multidisciplinary approach to all organ systems that may be affected by this disease. In severe cases, prompt
transfer to a burns unit is necessary in order to decrease both morbidity and mortality. While systemic
corticosteroids should be avoided in the management of SJS, IVIg should be considered early in the disease,
even though its efficacy has not been definitively established. Patients at risk of or those previously afflicted
by SJS should receive counseling on the importance of completely avoiding responsible agents or similar
compounds. Furthermore, physicians and health care workers are encouraged to improve documentation of
offending agents in patient charts in order to minimize the risk of repeat outbreaks of the disease.