The most interesting case I had this week

[Kaisu]

I was on the last day of a 72 hour shift and had finally gotten to bed. It was around 10:30 PM. I get wakened by my partner less than an hour later telling me I have been assigned to an emergent transport into Vegas. It's a big city hospital with burned out cynical staff, tough as nails triage nurses and some of the best and most economical patient care in the Cal-Nev-Ari region.

I had just finished a day where, among other things, I had fought it out with our medical director over Dr. 911 refusal to grant use of morphine in NTG refractory cardiac pain and wiped blood and tears out of the eyes of an alcoholic combat vet with serious facial trauma and head injuries. I was looking at a minimum of 4 1/2 hours on this transport.

The transfer documents said 29 year old male - chief complaint skin rash. WTF? Skin rash ? - this is emergent at almost midnight?.

Pick up our patient. He is calm, alert and orientated and very pleasant. He has a swollen appearance, a yellowish cast to his sclera and a red skin rash over most of his body. He has received morphine for pain ( rated 7/10 prior to treatment) with a good response to the pain medication. Other meds/Tx include a 1000ml NS bolus and 40 mEq potassium infusing via pump. The patient's skin is sloughing off his face and ears and he apologizes for his appearance. He is ashamed that his skin is coming off and shows up on his black t-shirt. The patient's 2 year old daughter is asleep on the floor in the corner of Dad's ED room, his spouse and 4 year old son are anxiously waiting in visitor chairs at bedside. I get a hinky feeling about this man. Although hemodynamically stable, and in no apparent distress, he presents to me as the sickest patient I have seen this shift.

We get a boatload of paperwork sorted out and load the patient. I set him up on the monitor, verify the patency of the IV, make sure the infusion is infusing, make sure the patient is comfortable. He says his pain is only a 3/10 or so and tells me that this is the first time all day he is warm. ( OK -no air conditioning for me I will sweat it out with him here in the back). I dim the lights and off we go. He falls asleep and I read the transfer documents in the light of the alaris pump.

Patient was being treated for a sinus infection. He had been on bactrim for about 14 days and had come into the ED with personal vehicle complaining of the painful rash. Liver enzymes off the chart. Diagnosis - Stephen Johnson Syndrome and hepatitis. Now, I blew the EMTs away yesterday because I knew what Guillam Barre was, but Stephen Johnson Syndrome? This had not been covered at medic school. I went to a pretty good school, and that was a clue that it had to be a relatively rare condition.

The patient wakes up and is talkative. I start eliciting details. His only medical history was asthma that had begun in his early teens and "gone away" by his late teens. Hmm.. clue 1 - reactive airway disease.

Clue 2 - allergies to penicillin and codeine - hmm.. auto immune system issues....

Hx of current illness - The patient has a history of sinus infections. He tells me that he has had one a year. In the past, they had been treated by common antibiotics with no problems. This year, the patient had moved and had a new doctor that prescribed bactrim. Shortly after beginning the course of treatment, the patient began experiencing fever, chills and increasingly severe headaches. He had complained of these symptoms to his doctor. His complaints had been dismissed as part of the course of the sinus infection. Finally, about 3 days prior to his entry to the ED, the patient had stopped the antibiotic. He had done this unilaterally, intuitively understanding that bactrim was part of the problem, not the solution.

The patient tells me that not only is the skin sloughing off his face and head, it is coming off his genitals. He has blisters in his mouth that make it impossible to eat and painful blisters on his feet that make it difficult to walk. He tells me he can't figure out which he wants first when he recovers - a big plate of macaroni and cheese or a huge steak. We discuss the merits of each, finally concluding that he wants a huge rare sirloin with a side of macaroni and cheese. I have this horrible intuition that the man may never get to this meal.

We spend the rest of the transport enjoying my partner's frustration with the idiots that drive slow over Hoover Dam.

Nice man - dreadful condition.

Thanks for letting me share.

[sjslennon]

SJS technically is a skin rash, but the real thing (like what you describe) is treated like a burn, often in a burn unit. Were you transporting to a burn center?

It is often from a drug, sulfas and pcns being common causes, as well as some anticonvulsants. It can also be caused by an infection or simply be idiopathic.

It exists as part of a spectrum. SJS is also known as erythema multiforme major. There is an erythema multiforme minor, which I see a few times per year. These we send home with close follow up. The other end of the spectrum is toxic epidermal necrolysis. You can see pictures of all of these rashes (plus some other gnarly pictures) at http://www.dermnet.com/moduleIndex.cfm?moduleID=22.

Be concerned for any pt with sloughing skin. As a rough general rule, the % BSA sloughing is the mortality rate.

Neat case! Thanks for sharing.

[ERDoc]

SJS is a rash, in the same way that a ruptured anuerysm causes a little bit of bleeding. Here is a good article from emedicine.

[firedoc5]

I remember listening to a conversation about SJS and I think I've only seen it with pts. in ICU. I remember the skin sloughing.

I can see this being treated as if it was a burn. The infection possibilities being very high as a burn.

I'm going to have to study up on that later. You've got my curiosity up. Thanks for sharing.

[Kaisu]

SJS is a rash, in the same way that a ruptured anuerysm causes a little bit of bleeding. Here is a good article from emedicine.

EXCELLENT article. Thank you for providing that. Once again, another area of ignorance exposed and rectified. This is an amazing job.

[Eydawn]

I'd read about this as an adverse effect of anti-convulsants before, but never this much in depth. Great article, doc! Here's hoping he makes it and recovers, Kaisu.

Isn't it amazing what can happen to the human body for no apparent reason? I think this would be fascinating to study in some sort of mammalian model... I bet it has something to do with cell death pathways (just my uneducated Monday morning guess).

Wendy

CO EMT-B

[Doug]

I have seen a person like this before, reaction to anti biotics. In the case I saw the person had a smell not entirely unlike a burn pt. and in fact he was being transfered to a regional burn center. Interested in what kind of facility your pt. was being transfered to. Is this topic open to presenting our own interesting cases? Because I've got one but don't want to hijack somebody elses thread.

[Kaisu]

By all means respond with your interesting case. The transfer was ED to ED but I believe the eventual floor was ICU. The facility is not a burn center.

[CBEMT]

I once transported a young girl with early SJS. She spent at least a month in the PICU and another two months or so inpatient. The peds resident on the transport said SJS always gets worse before it gets better.

[Arizonaffcep]

I got to see a person with SJS on the burn unit in St. Mary's in Tucson (when it was still considered a burn center). According to them, SJS, TENS and other similar disease processes are best treated in a burn center because of the loss of the integumentary system. Quite an interesting disease...although definitely not pleasant.