The skin of the patient with SJS is extremely fragile; lesions may develop easily and just as easily rupture to produce a new skin barrier disruption. Anesthetists must take extraordinary care when positioning the patient and padding bony prominences and pressure points. The best padding for the patient with SJS is to use gelatin-filled pads that maximize the dispersal of pressure. In addition, the patient with SJS should have his or her skin protected from the shearing forces the blood pressure cuff can produce, which will exacerbate skin barrier disruption. Similarly, securing intravenous catheters, endotracheal tubes, and protecting the eyes with tape can be detrimental to the patient with SJS because removal of the tape can also remove the underlying skin. Intravenous lines may be best secured using a suture rather than tape, followed by covering the site with a sterile gauze dressing. The endotracheal tube can be secured using any of the commercially available devices. The patient’s eyes can be protected by placing lightly moistened gauze pads on the closed eyes then wrapping the head with gauze. The anesthetist should also take similar care when removing the electrocardiogram pads and should strive to minimize the shearing forces produced.

Intravenous access is a critical need for the patient with SJS for both anesthesia administration and fluid resuscitation. Central venous access is discouraged. However, if larger fluid volumes are required, it is obviously an avenue to be cautiously explored after weighing the risks versus benefits. The patient will have nutritional needs similar to those of a patient with burn injuries and may need to receive parenteral nutrition on arrival at the operating room. It is important that the parenteral nutrition be maintained throughout the duration of the anesthetic. The patient also may be receiving enteral nutritional support critical to the healing process. Although it presents the risks of a full stomach, the anesthetist should allow the supplementation to continue, possibly at a slower rate, until the patient is delivered to the operating room to be prepared for anesthesia. Induction of general anesthesia must, of necessity, follow the classic rapid sequence induction precautions.

The patient with SJS frequently has significant involvement of the oral cavity and oropharynx, and may present with major laryngeal edema. These tissues will be almost astoundingly friable. Hemorrhagic bullae form with seemingly the most simple contact; direct laryngoscopy, endotracheal intubation, oral and nasal airway insertion, placement of an esophageal stethoscope, or passing an orogastric or nasogastric tube may produce and rupture these bullae and cause immediate severe airway compromise. The use of a laryngeal mask airway is not strictly contraindicated in the patient with SJS, but its use must be very carefully considered, especially in the patient with extensive oral cavity involvement, despite the reduced use of positive pressure ventilation it can afford.

The involvement of mucous membranes in the patient with SJS can extend further down the tracheobronchial tree, resulting in development of visceral pleural blebs. These blebs are likely to rupture, producing pneumothorax. For this reason it is desirable to avoid positive pressure ventilation where possible. In cases where positive pressure ventilation is unavoidable, it is advisable to prolong the inspiratory phase with reduced peak inspiratory pressure by selecting a pressure-controlled method of ventilation available on most late-model anesthesia machines. At the same time, the lowest effect peak inspiratory pressure should be selected as well. These measures will help minimize the potential for bleb rupture. In addition, because of its rapid diffusion and expansion, nitrous oxide should not be used, especially if tracheobronchial tree involvement and/or pleural bleb formation is suspected.

Because of the patient’s high degree of pain, potent opioid analgesics may be in use with adequate or inadequate pain relief. The anesthetist should anticipate that the patient has developed tolerance to the opioid analgesics and should be prepared to administer large doses of opioids to achieve seemingly minimal results. No specific anesthesia drugs or anesthesia techniques have demonstrated clear superiority for the patient with SJS. Infiltration of local anesthetic must be undertaken cautiously, with due consideration for the condition of the patient’s skin as well as the increased potential for infection. Drugs that have been implicated in the development of SJS, such as barbiturates, should be avoided. Propofol has been used safely to anesthetize patients with SJS, as has ketamine. Both can be used as the sole anesthetic agent if necessary. The anesthetist must also consider the route of elimination for anesthesia-related drugs when planning the anesthesia care for the patient with SJS. In particular, the anesthetist should avoid potentially nephrotoxic drugs and those eliminated via the kidneys when renal involvement has been demonstrated.