Stevens-Johnson syndrome

Learning objectives

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

One to three days before a rash develops, early signs of SJS include

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

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

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. medications (e.g., corticosteroids)

Maintain skin and mucous membrane integrity
- Minimize handling and transfer of patients to prevent further epithelial damage or rupture of bullae
Safe airway management
- Mucous membranes of the respiratory tract and pleura may be involved
- Ulcers in the nose, oropharynx, trachea, and bronchi increase the risk of hematemesis
- Avoid manipulation and trauma to the oral, tracheal, and laryngeal mucosa (lesions are often present)
- Avoid desquamation of the skin on the face during mask pre-oxygenation
Difficulties getting venous access, application of monitors and anesthesia face masks, airways, laryngoscopy, and intubation
- Establish access over areas of normal epidermis
- Use invasive BP monitoring
- Use one smaller than the standard size for laryngeal mask airways
Adhere to strict aseptic precautions at all stages (immune-compromised state and susceptibility to respiratory infection)
Induction with etomidate or ketamine could provide better hemodynamic stability when compared to propofol or thiopental
Non-depolarizing muscle relaxants are often used if muscle relaxation is necessary to avoid succinylcholine and the associated risk of hyperkalemia
Fluid and electrolyte replacement
Avoid hypothermia
- OR temperature 28°C
- Warm i.v. fluids
- Water mattresses/forced air-warmers
Adequate postoperative analgesia

Proper preoperative evaluation, identification of the precipitating agents in order to avoid them, a continuation of preoperative medications, and other immunosuppressants or steroids is essential
Extreme care should be taken while transferring patients with fresh lesions
Patients with involvement of the respiratory tract and pleura require special attention, including the application of a face mask, laryngoscopy, intubation, and suction with extra lubrication when general anesthesia is chosen
Anesthetic drugs can be safely used in SJS patients