Stevens-Johnson Syndrome

00:02 Stevens-Johnson syndrome. So let's talk about that for a moment.

00:06 Essentially, Stevens-Johnson syndrome is most often a drug-mediated disease process that lies on a spectrum with, at one end, erythema multiforme, which can be caused by either medications or sometimes infections, then Stevens-Johnson syndrome, medications or sometimes infections, then Stevens-Johnson syndrome, It is a T cell-mediated attack on keratinocytes. And again, if the attack is on the keratinocytes themselves, rather than the basement membrane, you're going to expect very thin-walled bullae, which are very likely to rupture.

00:39 You're not going to have those tense bullae. As I mentioned, most often, it's caused by medications, but 70% of the time, though, occasionally, its associated with mycoplasma or other types of infections in < 30% of cases.

00:54 SJS, unlike something like bullous pemphigoid, you're definitely going to have some constitutional symptoms: fevers, myalgias, malaise, etc.

01:04 nd this is a fairly rapidly progressive disease, as well.

01:07 This isn't something that's going to be indolent and take months to develop.

01:11 This could really occur within a matter of a few days to a week.

01:15 So, rapid progression of erythematous macules, then erythroderma, which means total body redness, and then your bullae are going to form, you're going to start seeing those mucosal lesions, etc.

01:26 In this case, because that skin, the wall of that blister, is so thin, Nikolsky sign will be positive.

01:33 Just rubbing your thumb along an area of skin that appears to be affected is going to lead that skin to peel right off.

01:41 90% of cases are going to have mucosal involvement, whether it's the oral mucosa, the lips, the conjunctiva, or the genitalia.

01:49 And as you can imagine, with a disease that involves such an extensive area of skin as well as mucosal areas with all these systemic symptoms, it does have a pretty high morbidity and mortality associated with it.

02:00 You're very susceptible to infections, electrolyte imbalances due fluid shifts and being unable to hold on to all the water in your body, hypovolemia, and potentially, septic shock.