Tube feedings are a critical component of care; TPN is used only to supplement enteral nutrition in cases of an ileus. A euglycemic state is maintained, using insulin if necessary. Pain relief, sedation and maintenance of normothermia are also important. Psychological support is essential. This is a devastating illness that occurs after the innocent use perhaps of an antibiotic for an ear or urinary tract infection or the initiation of antiseizure therapy. Patients feel clearly betrayed by doing what they were told to do or thought was right and ending up with this problem.
Involvement of ophthalmology and dermatology at the onset of the disease is important. Skin biopsies are required both to establish the diagnosis and exclude other diseases. Patients can get conjunctival synechiae and the ocular complications are both a late and a devastating problem, with some patients experiencing visual loss.
The topical therapy is very much like a burn. We do hydrotherapy, cleansing and debriding of loose tissue. We like not to use silvadene because it is a sulfa-based compound which may reactivate the process. Instead we use 0.5% AgNO3 topically. Sometimes we use sheets of Xeroform, a vaseline impregnated gauze with bismuth in it that has some minor antibacterial properties. This will remain adherent until the underlying skin reepithelializes, then it spontaneously separates.
There have been uses of synthetics, like Biobrane, a nylon mesh which has been populated with growth factors and, in some cases, cultures of neonatal fibroblasts derived from foreskin. These both serve as a biological agent to provide growth factors topically and to seal the wound. Additional biologics are xenograft (pig skin), amnion and allografts (cadaver skin). There are also some composites of both biologic and synthetic agents used to cover the defects created by the absence of skin.