STEVEN JOHNSON SYNDROME,cause and effect,and prognosis for full recovery?

Question

Is anyone familiar with this horrific disease,know the best Doctor to
deal with it,as well as the best atty.to deal with the "aftermath"?My
wife is almost blind from it.After two years of skin grafts,and much pain,the eyes are our biggest concern now.She's so beautiful,she was a Playboy centerfold,and she can't see herself now and needs
the BEST eye specialist around. I'm lucky to have her still! Please
help if you can! Thanks,Harry Beam Dallas,Tx.

Answer 1

Stevens Johnson is a bizarre phenomenon that is not well understood. In general, it is not an allergic reaction, although that's what most people think. The general concept is that as a side effect of some medication adminstration, the patient undergoes a toxic response and "desquamates" or loses the epidermis over regions of the body totally less than 30% of the total body surface area. If the loss is more than 30%, then the terminology "Toxic Epidermal Necrolysis Syndrome" or TENS is used. Any medication could be implicated in this kind of reaction, but some of the more commonly associated are dilantin (an anti-seizure medication) calcium channel blockers (blood pressure medication) and sulfa-type antibiotics (the most common being bactrim). This reaction is pretty rare, but I see it fairly frequently because I am a surgeon at a facility with a large regional burn center. These types of patients are often best served at a burn center.

One of the more common errors in treatment is that unfamiliar providers will see what they believe to be an allergic reaction and will administer steroids. Steroids can blunt the immune response, and when the patient soon desquamates, they have large areas of open skin as a portal to infection.

It sounds like your wife has really been through a lot, and she is clearly well past the initial phase of her injury. The issue with the eyes will best be addressed by an ophthalmologist who can examine her directly. The question to ask them as they are examining her is "what would be the possible effectiveness of a corneal transplant?" Corneal transplants are very effective in selected patients, and this is also something that comes up at burn centers, as thermal injury to the eye can coagulate the cornea, rendering it opaque.

Answer 2

Please see the webpages for more details on Erythema multiforme.