Invictus Reviews

A fever and a Rash

• Mel Herbert

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Stevens-Johnson Syndrome presents a critical dermatological emergency characterized by mucosal lesions and diffuse rash with high mortality if untreated. Recognizing this condition requires understanding its presentation, pathophysiology, and immediate management strategies to improve patient outcomes.

• Case presentation of 20-year-old female with fever, mucosal lesions, and diffuse rash
• Mucosal involvement is the key diagnostic feature of Stevens-Johnson syndrome
• Common triggers include sulfa drugs, anti-epileptics, NSAIDs, and infections like mycoplasma
• Positive Nikolsky sign where skin cleaves and sloughs off when pressed
• SJS affects less than 10% of body surface area while TEN involves more than 30%
• Treatment includes supportive care, fluids, nutrition, and controversial steroids
• IVIG, plasmapheresis, and TNF blockers may be beneficial treatment options
• Approach treatment similar to severe burns with non-adherent dressings
• Secondary infections may require antimicrobials, but not as initial treatment
• Eye involvement requires artificial tears and careful monitoring

Join us for our upcoming Encore program focused on preparation for the new oral exam, followed by our comprehensive Invictus board review course.


Speaker 1:

Hey, little heads up. This is very visual. There's a link for the YouTube thing, but there's still something useful in this audio. So here we go. Hey, people of the Invictus free podcast, want to do some updates. We've laid down a lot more video, a lot more questions, a lot more summaries. It's happening soon, and actually ahead of the release of the full Invictus, we're doing Encore, which is for the oral exam. So that's going to come out in the not too distant future, much shorter, but it's all about preparation for that new oral exam, which is very different from the old oral exam.

Speaker 1:

But today what I want to talk about is some dermatology, because I hate dermatology. We've been talking about some rashes on the show. Let's talk about another thing. Let me give you a case. It's a 20-year-old female. She comes in and she's had fever for a few days, some aches and pains. She's got some mucosal lesion and just sort of this diffuse rash that developed this morning and which was the thing that made her come in. When you look at her, you see that she doesn't look too well. She looks a little bit toxic and, sure enough, she's got some lesions in her mouth, in her mucosa. She's got some redness of her eyes, she's got this sort of diffuse rash, and what do you think this could be? It's very nonspecific, right, and that's the point. It's very nonspecific.

Speaker 1:

But one of the things that's really concerning, obviously, is the fact that it is involving the mucosa, and anytime you've got a diffuse rash that involves the mucosa you've got to think about Steven Johnson syndrome. And when you think about Steven Johnson syndrome you think about drugs and you think classically, the thing is sulfur drugs, but a whole lot. But anti-epileptics can do it, non-steroidals can do it, there's a bunch of drugs that can do it, and there's also infections like mycoplasma viruses. So there's a bunch of things that can trigger this, which then probably turns it into an autoimmune disease where you start getting cleaving of the skin. And so one of the classic things with Steven Johnson syndrome and toxic epidermal necrolysis, which is really just the severest form of the disease, is that you can have a positive Nekholzdi sign. So you, over time, might develop bullae, and those bullae, as you press on them, they can sort of cleave that epidermis and get bigger and bigger and bigger, as you can see in some of these pictures. It can start off as these sort of targetoid-like lesions, then bullae and then, as if it's a really bad form, you can slough all this off and it looks like and acts like and should be treated much like a burn. It can also affect the eyes, it can affect the mucosa all over the body and even today it has a significant mortality in the severe form.

Speaker 1:

So classically toxic epidermal, toxic, slow down, toxic epidermal necrolysis is when more than 30% of the skin is involved and Stephen Johnson's when 10% or less of the skin involved and then there's that place in between the 10 to 20 to 30% where it's sort of an overlap between the two. The diagnosis is classically made just sort of on the history, the physical. Yes, you can do skin biopsies and kind of work out with the histology what it might be. The differential diagnosis is pretty broad. But if there's no involvement of the mucosa it is unlikely to be stevens johnson syndrome or toxic epidermal necrolysis and that might come up and probably will come up in the question. So you can have a number of different patients that present like this. But if there is not mucosal involvement it's not likely to be Stevens-Johnson's or TEN and if there is involvement of mucosa then it is much more likely to be that.

Speaker 1:

What is the treatment? The treatment is supportive fluids. Nutrition Steroids are very controversial. There's a timing thing here. Maybe if you get them early it can reduce the disease and maybe if you give it later it can make it worse. So very controversial. You do that in association with a dermatologist. What else can we give for these people? We can certainly give IVIG. That appears to help. Then there's stuff like plasmapheresis and some of these tumor necrosis factor blockers which may be helpful. But overall, mostly in the immune department, it's supportive.

Speaker 1:

And also the eye involvement can be quite severe. So you're going to put in artificial tears and if there's big time slothing of the skin you might want to be trimming that up and putting on non-adherent dressings. And if there appears to be secondary evidence of infection you might give antimicrobials. But you don't do that up front. That can sort of complicate the picture and again, think about this as like a severe burn. You know that's how you would treat these people.

Speaker 1:

So there it is Stevens-Johnson syndrome, toxic epidermal necrolysis caused by a lot of drugs. Some infectious disease can do it as well. Mucosal involvement is the key thing. Targetoid lesions, initially positive Nikolsky's sign All right.

Speaker 1:

So I'll do some more of these and this is all as we ramp up to do the big giant dump of what is going to be on call, which is for the oral exams, and then Invictus, which is our big board review course. So you're going to get a huge dump of exams, and then Invictus, which is our big board review course. So you're going to get a huge dump of content. But then, unlike anybody else, we're going to continue to do live events and question banks and add to this and have the discussion and make it a community so that you can crush your exams, but also in-service exams and final exams. But also you might want to continue to be a part of this community for your entire career, because I believe I've always believed that good emergency care requires that you do continuous board review, that you learn this stuff over and over and over again, because anything can come through that door. All right, herb it out.