Survival should be almost uniform. We had 1 death in a patient who developed acute respiratory distress syndrome, was treated with ECMO and ultimately died. Complications include loss of vision; this was in one patient who required a corneal transplant. Several patients have had severe and persistent hypopigmentation.
The treatment in general is either ICU or burn unit admission. You need to establish stable IV access with either a CVC or PICC line. Sedation and cleansing with debridement of the loose skin is done and dressings are applied. Different from a burn, this happens in stages - it doesn't all happen on day 1. So there is a variable change in terms of their wound care needs. Because of that the wounds require daily inspection.
A feeding tube is placed, because it is difficult for these patients to swallow, as well as a Foley catheter, because there can be urethral involvement also. Anywhere there is squamous epithelium there can be disease involvement. Skin biopsies are done at the margins of the wound times two to support the diagnosis.
The wound care is:
- Xeroform for areas of confluent sheets
- a topical anti-inflammatory agent, like Avosil, for areas where there are blisters but intact skin
- Aquaphor for the lips
Antibiotics are culture driven. Nutrition is targeted at twice their resting energy expenditure, with enteral being the preferred route.