Steven Johnson Syndrome/toxic epidermal necrolysis (SJS/TEN) is a very severe reaction, most commonly triggered by medications, that causes skin tissue to die (necrosis) and detach. The mucous membranes of the eyes, mouth, and/or genitals are also commonly affected. SJS and TEN were previously thought to be separate conditions, but they are now considered part of a disease spectrum. SJS is at the less severe end of the spectrum, and TEN is at the more severe end. It is considered SJS when skin detachment involves less than 10% of the body surface and TEN when skin detachment involves more than 30% of the body surface. People with skin detachment involving 10-30% of the body surface are said to have “SJS/TEN overlap.” All forms of SJS/TEN are medical emergencies that can be life-threatening.
–NIH
Skin conditions and treatment are tricky in general and Steven Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) is no exception. I briefly wrote about it in complications and at the time, I rarely prescribed lamotrigine just because I was primarily seeing kids (or people who can’t communicate this issue well…). However, recently it’s been one of my favorite medications for adults mainly because it’s not high maintenance like lithium. So of course, I had to have one with a reaction despite taking super precautions. I also had a patient with a reaction to valproic acid so I’ll go over a few points and case studies.
The patient (JB) has been on lamotrigine for the last 2-3 months and requested to increase the dose to 200mg TID, instead of the BID. At first, I was going to say it’s not recommended, but the pt. was already doing this suggesting his mood is much better. In general, I start meds at the lowest so a pt. could increase as tolerated if needed and discuss side-effects or symptoms before and after starting medications because humans forget stuff all the time…this is the main reason why I don’t prescribe it to kids.
Like with any med, the higher you go, the more likelihood for side effects, and technically it’s okay if it wasn’t for serious reactions. I have patients on higher dosages than what’s approved just as long as they are aware of the risks and not having major S/E.
Despite all this, JB called me a week later after a f/u to ask about an increasing rash. It was already on his chest and arms, but it wasn’t until it was on his face that he remembered “the rash”. We were on the phone but I assumed that if it was major, the person wouldn’t have forgotten during the recent med check, but still gave a disclaimer to go to the ER. However, the pt. didn’t want to go to the hospital since it was only a rash, but I discontinued the med and changed to another mood stabilizer. Remember, it’s best to taper a mood stabilizer (to not relapse into mania, i.e.) but depending on the severity of the reaction, it can be stopped immediately.
Additional Precautions
I told JB to take diphenhydramine 50mg QHS, use topical steroid cream as needed for the affected areas, increase the fluid intake to a liter (like a bootleg ICU), and limit or avoid sun exposure. I DON’T RECOMMEND THIS but I have patients that are defiant. Sidebar: I tell people to avoid the sun anyway with mood stabilizers because they’ll assume it’s SJS when it’s actually a sunburn and these medications can affect the skin, cause light sensitivities… and overall SJS’s etiology is unknown, but it could be other differential diagnoses including; SSSS, autoimmune bullous diseases, bullous fixed drug eruption, erythema multiforme, thermal burns, phototoxic reactions, and TSS (-Core EM).
Another sidebar: there isn’t an outpatient plan of care for any SJS reaction, they need to go to an ER – a rash ulcers/blisters/swelling in the chest area will compromise the airway, but mild skin reactions can be treated and if it doesn’t clear up then they should definitely go to the hospital. If treated early enough, it shouldn’t be more issues and thankfully my patient was okay. (Make sure you document especially when a patient refuses to seek immediate treatment)
I have another patient that I wanted to taper off lamotrigine due to a skin reaction but she didn’t want to stop it (she was worried about her mood). She agreed to once/day dosing and to start another mood stabilizer at the same time, and after increasing the 2nd mood stabilizer, she’s doing much better. She believed her skin reaction was r/t the warmer weather/sun, which can definitely be true and lamotrigine can cause “benign rashes” (-drugs.com). Some people are just hypersensitive to meds, life, the environment, mental health, etc. thus I try not to make too many sudden changes if tolerated. Overall, good communication is key and I try to make myself easily accessible, especially if there’s a reaction involved.
Depakote (VPA) Rashes
I inherited a teen on VPA and his mother mentioned how his feet are becoming more discolored. I usually go over side effects even if someone else prescribed the med because things can be easily missed. However, despite the teen being on the VPA for months, this was a new reaction that I assumed VPA was more than likely the culprit. The patient was already on another mood stabilizer so we just tapered the VPA, to see if the rash would resolve and since it did, we just discontinued valproic acid. Also, don’t forget to document these reactions as a med allergy.
Again, taper as tolerated to not cause a relapse in mania/psych symptoms. I usually tell patients on mood stabilizers to use sunscreen or limit sun exposure and drink extra water. These meds increase photosensitivity and getting sunburn can cause blisters, skin peeling, or make it hard to differentiate between an initial med reaction or something serious.
Other Cases
Burns from the Inside Out: this is an interesting case because his main initial SJS reaction was in his eyes. I wanted to quickly note with this article how the side effects of lamotrigine can also include eye issues, but the person also mentioned how he had blisters in his mouth and throat –and that’s always a major red flag! Some additional ocular S/E with lamotrigine but this also applies to most mood stabilizers:
- Very common (10% or more): Diplopia (28%), blurred vision (16%)
- Common (1% to 10%): Vision abnormality, nystagmus, photosensitivity, amblyopia
- Uncommon (0.1% to 1%): Abnormality of accommodation, conjunctivitis, dry eyes, photophobia
- Rare (less than 0.1%): Lacrimation disorder, oscillopsia, ptosis, strabismus, uveitis, visual field defect (-drugs.com)
I wrote something about vision issues with psych medications but want to mention how I just do a general statement with patients to report back ANY side effects, some things are truly unknown with these medications. They don’t even know how most of them work. I have an older patient who had ringing in his ears until he had to get hearing aids and I talked about how VPA can cause this reaction. He was upset that the provider who put him on the medication for the past year didn’t mention this side effect, but I told him it’s somewhat theoretical. However, with the decreased dosage of VPA, he was able to reduce this buzzing, and he still sees his psychiatrist. His hearing loss was the main source of his depression, but was seeing me for therapy and thankfully he was finally able to perk up. So again pay attention to the odd/rare side-effects especially if the problem is not their baseline. Sidebar: if another provider made whatever mistake, don’t throw them under the bus… you don’t know the full story nor is it your job. If it’s something serious, follow the chain of command or use appropriate means. I just personally try to limit or avoid disrupting a person’s continuity of care with others and guide/educate the patient as needed.
Another Incident
The last case is Khaliah’s heartbreaking story. Her blisters started 2 weeks after starting lamotrigine and the claim is that she received the wrong dosage. They don’t mention the dosage and that’s frustrating, so I’m going to assume that it probably wasn’t the lowest or the highest dose, which means it’s hard to hold the provider or the pharmacist liable. Depending on how severe her mood symptoms were or her health history that could be a reason that the doctor started at a higher dosage. If the doctor warned about SJS, it could also be a matter of the doctor’s word against hers and that will also be difficult to prove vs. an act of nature… Not sure of the outcome of the case, but the moral of the story is always to start low and then titrate. Once again SJS is rare compared to the millions of people who take these medications but it’s extremely serious.
Several EMRs have lamotrigine available at 25mg (or the lowest) then the next dose is 100mg, and then 200mg. I usually prescribe 25mg QAM (so they won’t be sleeping if there’s a reaction, but they can switch to QHS if it’s causing too much drowsiness) x2 weeks then 25mg BID after 2 weeks, and I’ll write out instructions but prescribe it as 25mg BID x30 days. I don’t use the starter packs because I want the patient to be mindful that the titration is r/t SJS risks -so they will have NO excuse to say, they didn’t know… If they have other psych symptoms, I’ll use different meds to target them. I understand doing one medication at a time, but it depends on the clinical picture, etc.
In Conclusion
I’m not a dermatologist nor do I try to play one, and good luck finding one or getting an appointment. Unfortunately, I usually feel like I have to be more than my scope of practice so my go-to website for anything about the skin is DermNet NZ and they have pics! This is not for diagnosing but weird stuff happens in psych and if it’s skin-related, this website will talk about it… however, know when something is an emergency and will require intensive care. If you pay attention to the world, most places don’t even print up paperwork anymore (they are MORE dependent on YOU aka the provider) so I have to keep giving my same PSAs and speeches, but in general consider additional ways to avoid liabilities and complications. As you can see these hospitals, clinics, facilities, etc. are also trying to avoid liabilities and will use anyone else as the scapegoat. Think about the way you practice and see how you can further protect the patient AND especially yourself. The last photo here since it’s somewhat graphic:
