We’ve all been there – that dreaded middle of the night page/call from the ER. Three simple words that send us spinning: “Rule out TEN.” Have no fear though, because the skin sloughing savoir is here. Inpatient dermatology service director Dr. Helena Pasieka joined JDD podcast host Dr. Adam Friedman to discuss her recent research that elucidates opportunities to prevent offending agent re-exposure (aka first time shame on you, second time shame on us) in patients with a history of SJS/TEN. Simple steps can be taken to improve communication and prevention. Even more importantly, Dr. Pasieka walks us through her tried and true approach to that r/o call, key findings to guide your decision making, and what to when.
Dr. Aaron Fong, PGY-3 dermatology resident at George Washington University School of Medicine and Health Sciences, shares key takeaways from Dr. Pasieka’s podcast “Don’t Go Zero to TEN: Identifying Gaps that Lead to Drug Re-exposure in Toxic Epidermal Necrolysis” and her JDD article titled “Recommendations for Prevention of Drug Re-Exposure in Toxic Epidermal Necrolysis”.
Dr. Pasieka was inspired to write this case series as she sees many SJS/TEN patients at her metropolitan referral center, and she works closely with the burn intensive care unit there. She noticed that several patients with suspected SJS/TEN was due to drug re-exposure, so she wanted to investigate further.
She identified several different reasons why re-exposure can occur. Factors that contribute to this phenomenon include polypharmacy, multiple prescribers, advanced age, medical illiteracy, retention of discontinued medications, and self-prescribing.
Dr. Pasieka emphasized the importance of drug calendar or timeline to help identify the offending agent.
She notes that physicians should properly counsel their patients on the importance of avoiding offending medications and the consequences of re-exposure. Patients should know that medications causing SJS/TEN should never be rechallenged, or they may risk a life-threatening reaction.
Dr. Pasieka suggests taking a more active role as a consultant in these cases of life-threatening drug reactions. Specifically, she advises other dermatologists to manually add medications to the allergy list instead of just providing recommendations and relying on the primary team to add them.
When entering an allergy to a patient’s chart, Dr. Pasieka recommends referencing the original dermatology consult note so it can be referred to in the future.
One key point Dr. Pasieka brought up was to keep checking and rechecking the drug history timeline. A patient’s story can often change, and sometimes they remember important details later. Family members can also a good resource to elucidate the drug history. One last pearl is to have a family member look through the patient’s home for all available medications. Persistence is often key to obtain a complete drug history.
Patients from outside the United States were overrepresented in this cohort. Clinicians should take care to discuss drugs with their generic names, as foreign brand names can be different.
Advice on How to Handle a Consult to Rule Out SJS/TEN
The classic timeline for SJS/TEN should be used to aid in the diagnosis. 10-14 days after initial exposure to the offending agent is most common for SJS/TEN, but it can be a minimum of 4 days.
For re-exposure to a medication, SJS/TEN can present within a few hours afterwards.
Patients with SJS/TEN feel very sick. They will often have flu-like symptoms, myalgias, sore throat, nausea, or poor appetite.
Dr. Pasieka states that acetaminophen is likely overrepresented as a potential etiology of SJS/TEN. The typical story is that patients start experiencing systemic symptoms due to SJS/TEN, take acetaminophen, and then develop a rash as their symptoms continue to progress. The timeline of the onset of symptoms is especially important in these cases to tease out whether acetaminophen was truly a culprit or taken after the systemic process had already begun.
In >90% of cases of SJS/ TEN, there are at least two sites of mucosal involvement. This is very important to help triage whether the consult is emergent or can wait to be seen. Mucosal sites include conjunctival, intraoral, perianal, urethral, and vulvovaginal.
One way to test if intraoral mucosa is involved is to ask the patient if they would eat something right now.
Examine all mucosal sites. Patients often do not disclose genital involvement.
Cutaneous involvement of SJS/TEN can be polymorphic, so it is hard to pin down the diagnosis based on morphology.
If the patient is complaining of skin burning or pain, we should have a higher suspicion for SJS/TEN. Pruritus is not as common and is more likely a feature of a morbilliform drug eruption.
Nikolsky sign can be helpful in determining the amount of skin involved and how much normal skin is left that can be saved.
Dr. Pasieka states that biopsies are not necessary to make the diagnosis with experience, and that she was trained to do the “jelly-roll” technique, where a thin detached skin sample is taken and prepared by frozen section. However, biopsies in cases for suspected SJS/TEN can be helpful for many reasons. Mimickers can be difficult to differentiate from SJS/TEN, and biopsies processed with H&E and direct immunofluorescence (DIF) can aid in making the diagnosis. Furthermore, biopsies are essential in medical-legal cases, and patients and families often want answers and can be upset if there is no biopsy to confirm the diagnosis. Doing a biopsy can be therapeutic for both the provider and the patient.
There is not a lot of quality data or a consensus on the treatment options for SJS/TEN, and it is difficult to study due to nature of the disease.
Dr. Pasieka references the two class A, evidence-driven interventions for SJS/TEN: identify and stop the offending agent and perform exceptional skin care.
Early intervention by ophthalmology is important to prevent and reduce eye complications.
Dr. Pasieka decides her first-line agent based on the patient’s comorbidities (HIV, SLE, autoimmune disorders, etc.). She still uses steroids and IVIG, and she has been moving towards using cyclosporine more often, especially in younger patients with normal renal function and blood pressure.
On a closing note, Dr. Pasieka emphasizes the importance of a medical alert bracelet in these patients.
Make sure to tune in and listen to the podcast here.
Words From The Investigator
Dr. Aaron Fong had the opportunity to ask Dr. Pasieka a few questions on her advice to residents and the one book she thinks everyone should read.
What should we all do in our practice to prevent or minimize drug re-exposure?
We should encourage all patients with SCAR should be advised to get a medic alert bracelet and update their information in their smart phones. We should educate the patients’ family members and supportive individuals as well. We should encourage the family to go through all the medications in the patient’s home to remove any that are allergens or cross-react – often times I ask them to bring in all bottles for my review. We should be advising folks to know that the brand names of medications are different in other countries – and encourage the patients and their families to always know the generic names of their medicines, which is useful both here and when traveling. We should encourage patients to speak to the pharmacist when picking up a new medication, and they should tell them of their prior diagnosis and the agent that caused it (this is where a medic alert bracelet is very helpful – less to remember). Finally, patients have a poor understanding of what an EMR can do – They assume that information is transmitted between systems and between prescribers/pharmacies, but we all know that’s not true. I encourage all patients to call ALL doctors and prescribers personally and let them know that allergies need to be updated in their systems. Additionally, I ask them to do the same for all pharmacies where they are known to pick up medications.
What is one piece of advice you have for current residents and young dermatologists?
– Sorry, you get more than one… Haha.
- Residency is a bit like parenting… The days are long, but the years are short. Residency ends before you know it. Understand and try to accept that the more you can see and participatein now, the better dermatologist you will be for the remainder of your (hopefully long) career. Try not to gravitate too heavily towards the things you like in dermatology – you’ll have plenty of time to make a career out of that! Instead, embrace the things to which you don’t feel naturally drawn, so that you can strengthen any deficits. It’s like training weaker muscles in the gym! Try to make whatever sacrifices you can to get every bit of benefit out of this special training period – it’s over before you know it.
- Spend a bit of time reading and learning about personal finances for physicians. Because we are so focused on learning about our patients and perfecting our craft of medicine, we are neglectful of understanding the nuances of life and disability insurance, investing, payment of educational debt, and retirement savings and too many physicians find themselves in debt or as prey for someone else to “manage” these things for them – often at a high cost.
- Know what your riskscand liabilities are when you choose to supervise NPs and PAs in practice. There may be benefits to such an arrangement, but the responsibilities lie squarely with you as the “supervising doctor” for the treatment of your patients.
What is one book everyone should read?
I like White Coat investor for the basics of personal finance for physicians. I also love Bonnie Koo’s website (she’s a Board Certified dermatologist who does finance education) and she’s a wizz. Wolverton’s Dermatology Pharmacology book.
And in general literature: Kitchen Table Wisdom, Mountains Beyond Mountains, Cutting for Stone, Where Breath Becomes Air, Genius on the Edge… All so inspiring and amazing. Medicine is an art – it’s where science meets humanity. It will always be a wonder and a delight and an honor to participate. It’s no surprise there have been so many books written about it.
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