Learning objectives Describe Stevens-Johnson syndrome Understand the course of Stevens-Johnson syndrome Anesthetic management of a patient with Stevens-Johnson syndrome Definition and mechanisms Stevens-Johnson syndrome (SJS) is a rare, uncommon, and acute exfoliating disease of the skin and mucous membranes with systemic manifestations of variable severity SJS is a delayed-type hypersensitivity reaction in which SJS-inducing drugs or their metabolites stimulate cytotoxic T cells (i.e., CD8+ T cells) or T helper cells (i.e., CD4+ T cells) to initiate autoimmune responses The initial lesions of SJS are diffuse erythematous macules with purpuric, necrotic centers, and overlying blisters that may progress to skin slough, resulting in widespread superficial ulcers and loss of the epidermal barrier fluid and protein loss fluid and electrolyte imbalance with hypoproteinemia Signs and symptoms One to three days before a rash develops, early signs of SJS include Fever A sore mouth and throat Fatigue Burning, red eyes As the condition develops, other signs and symptoms include Unexplained widespread skin pain A red or purple rash that spreads Blisters on the skin and mucous membranes of the mouth, nose, eyes, and genitals Shedding of skin within days after blisters form Causes Infections (e.g., pneumonia and HIV) Drug-induced Anti-gout medications (e.g., allopurinol) Medications to treat seizures and mental illness (e.g., anticonvulsants and antipsychotics) Antibacterial sulfonamides (e.g., sulfasalazine) Nevirapine Pain relievers (e.g., acetaminophen, ibuprofen, and naproxen sodium) Malignancy-related Idiopathic Risk factors HIV infection Weakened immune system: Organ transplant patients, HIV/AIDS, and autoimmune disease (e.g., systemic lupus erythematosus) Cancer: Particularly blood cancer History of SJS Family history of SJS Genetic factors Complications Dehydration Sepsis Eye problems (e.g., eye inflammation, dry eye, and light sensitivity) Pneumonia Acute respiratory failure Permanent skin damage Multiple organ failure Treatment Treatment requires hospitalization, in an ICU or burn unit Stop nonessential medications stop taking SJS-inducing drugs Fluid replacement and nutrition Wound care Eye care Temperature management Medications Pain medications to reduce discomfort Medication to reduce inflammation of the eyes and mucous membranes (e.g., topical steroids) Antibiotics to control infection, when needed Other oral or i.v
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